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.

Effects of Fat Grafting Containing Stem Cells in Microstomia and Microcheilia Derived from Systemic Sclerosis.

BACKGROUND: Systemic sclerosis (SSc) is an autoimmune disease with multisystem involvement, dominated by a general fibrosis. The early stage of the disease is associated with progressive damage to microcirculation, particularly in the respiratory tract, the gastrointestinal tract and skin. The face assumes a typical appearance characterized by microstomia (reduction of mouth opening) and microcheilia (thinning of the lips). These conditions cause a considerable reduction in performance status of patients. We treated them by fat grafting, rich in adipose stem cells, and we evaluated through time clinical, functional and aesthetic evaluation of oral pathology associated with SSc. MATERIALS AND METHOD: From September 2014 to May 2016, we enrolled and treated seven patients in the plastic, reconstructive and aesthetic surgery clinic. Through time, we evaluated the following parameters: evaluation of mouth opening (maximum opening in superior-inferior and lateral directions) and lip thicknesses, both measured by doctors of the aforementioned operating unit; variation in the quality of life as perceived by patients according to the MHISS scale (Mouth Handicap Systemic Sclerosis); variation in severity of labial fibrosis assessed by microscopic analysis of pre- and post-fat transfer samples in the pathology clinic; safety of the protocol, according to the management of side effects resulting from the procedure; aesthetic evaluation, made by external observers and non-experts in the field, on pre- and post-operative photographs. RESULTS AND CONCLUSIONS: We reported satisfying results, both functionally and aesthetically, for all parameters except one, for which the sample size might have proven critical. These data should be a starting point for further experimental research and clinical trials. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Improving oral function and cosmesis in a case of Freeman-Sheldon syndrome.

Children born with the rare congenital condition Freeman-Sheldon syndrome (FSS) have a characteristic facial appearance: microstomia, a long philtrum, 'H-shaped' chin abnormality and 'pinched lips' in addition to extra-facial features such as kyphoscoliosis and hand deformities. Such children often have problems with oral continence and difficulties with speech leading to both nutritional and psychosocial concerns. Prompt correction through surgery is therefore important in effectively managing the condition. We report the case of a 7-year-old girl who presented with the appearance of scarred lips with difficulties in closing her mouth, a speech impediment and oral incontinence. Using a muscle-sparing technique to reconstruct the upper and lower lips, better oral continence was achieved alongside markedly improved speech and an aesthetically pleasing result. Taken together, this case aptly illustrates the benefits of lip reconstruction in improving function and cosmesis in patients with FSS.

Application of a custom-made inlay structure to a sectional maxillary complete denture for a patient with microstomia: a clinical report.

Prosthodontic treatment for edentulous patients with microstomia is a challenge because of the constricted opening of the oral cavity. This clinical report presents the prosthetic management of an edentulous maxilla in a patient with burn-induced microstomia. A sectional maxillary complete denture was fabricated. With the use of a custom-made inlay structure at the overlapping metal base along the palatal midline, the sectional prosthesis was successfully and easily inserted and provided adequate function in the patient's mouth.

Effect of orofacial exercises on oral aperture in adults with systemic sclerosis.

PURPOSE: To examine the effect of a home orofacial exercise program on increasing oral aperture among adults with systemic sclerosis (SSc). METHOD: Forty-eight adults with SSc were assigned randomly to the multifaceted oral-health intervention or usual dental care control group. Participants with an oral aperture of <40 mm in the intervention group received an orofacial exercise program, which included daily manual mouth-stretching and oral-augmentation exercises twice a day with a total of 6 minutes for 6 months. The outcome measure was oral aperture which was measured at baseline, 3-months, and 6-months intervals. RESULTS: A significantly larger increase in oral aperture for participants received the orofacial exercise program was found when compared to those in the usual care at 3 months (P = 0.01), but not at 6-months evaluation. Participants' adherence rate to the exercise program was low (48.9%). CONCLUSIONS: The orofacial exercise program intervention for adults with SSc and microstomia did not show significant improvement at 6 months. In addition to the low exercise adherence rate, insufficient frequencies, repetitions, and durations of the orofacial exercises may contribute to these results.

Dental care for patients who are unable to open their mouths.

There are a number of diseases and conditions that prevent the sufferer from adequately opening the mouth. The danger of inanition, malnutrition, chronic periodontal disease, caries, and abscessed teeth are very real to this population. Dental treatment issues include inadequate access to the oral cavity, inability to locally anesthetize mandibular posterior teeth, inability to gain access for traditional operative dentistry, and lack of clearance for most oral surgery procedures. The purpose of this article is to provide the reader with a discussion of the various conditions and then discuss the dental and anesthesia issues for this unique population.

Hallazgos orales en el síndrome de Rubinstein-Taybi: a propósito de dos casos / Oral findings in the Rubinstein-Taybi syndrome: concerning two cases

El síndrome de Rubinstein-Taybi es una alteración multisistémica genética que cursa con dismorfia facial, y retraso mental y del crecimiento. Este síndrome se caracteriza por tener los primeros dedos de los pies anchos, anomalías cráneo faciales con una nariz aguileña, estatura baja y retraso mental y motor, con diagnóstico radiológico y clínico. La prevalencia es desconocida y es relativamente raro. Las cúspides en garra aparecen con frecuencia localizadas en los incisivos. No hay un patrón hereditario definido y la recurrencia es bastante improbable. Se encontró que la causa es genética, mutaciones en el cromosoma 16p13 (AU)

The Rubinstein-Taybi syndrome is a genetic multisystemicdisorder that involves facial dysmorphosisand mental and growth retardation. This syndromeis characterised by having broad first toes,craniofacial anomalies with an aquiline nose, shortstature and mental and motor retardation, withradiological and clinical diagnosis. The prevalenceis unknown and is relatively rare. The talon cuspsappear frequently located on the incisors. There isno defined hereditary pattern and the recurrenceis quite improbable. The genetic cause was found, mutations in the chromosome 16p13 (AU)

Morphometric analyses of the mandible in prepubertal craniofacial microsomia patients treated with an inverted-L osteotomy.

To analyze changes in mandibular form associated with an inverted-L osteotomy and autogenous bone graft, preoperative, early postoperative (EPO), and late postoperative (LPO) lateral cephalographs of 14 children (mean age approximately 9 years) with unilateral craniofacial microsomia (CFM) were scanned and nine mandibular landmarks digitized. Average mandibular geometries, scaled to an equivalent size, were generated using Procrustes superimposition. Cephalometry, Euclidean distance matrix analysis (EDMA), and thin-plate spline (TPS) analyses were carried out on mean mandibular configurations. Cephalometric results showed increases in oblique mandibular length (approximately 9% on average, P < 0.05) and increased ramus height (P < 0.05). Similarly, using EDMA there were also significant differences (P < 0.05) between the mean preoperative, EPO, and LPO configurations. The most demonstrable EPO change in the mandibular configuration using EDMA was increased oblique length (approximately 11%). This improvement depended on ramus oblique lengths increasing by approximately 26%, ramus height increasing by approximately 25%, and mandibular body length increasing by approximately 5%. For TPS analysis, affine and nonaffine changes contributed to the total spline. In all three comparisons the affine transformation showed an antero-inferior rotation of the mandibular configuration. For nonaffine changes the EPO configuration indicated a supero-inferior stretch of the mandibular configuration. The nonaffine LPO changes maintained the supero-inferior stretch of the mandibular configuration. It is concluded that improvements in the lateral facial profile of CFM patients can be achieved using an inverted-L osteotomy, with little relapse approximately 2 years postoperatively.

Maxillofacial prosthetic rehabilitation of a midfacial defect complicated by microstomia: a clinical report.

Severe limitation in the oral opening, though an uncommon clinical presentation, makes gaining access to the oral cavity difficult for any dental procedure. This article describes the maxillofacial prosthetic management of a patient with a midfacial defect complicated by postsurgical microstomia. Intraoral and extraoral prostheses restored the patient's speech, dental articulation, mastication, lip support, esthetics, and anterior oral seal.

Measurement of facial soft tissue mobility in man.

OBJECTIVE: The assessment of facial mobility is a key element in the treatment of patients with facial motor deficits. In this study, we explored the utility of a three-dimensional tracking system in the measurement of facial movements. METHODS AND RESULTS: First, the three-dimensional movement of potentially stable facial soft-tissue, headcap, and dental landmarks was measured with respect to a fixed space frame. Based on the assumption that the dental landmarks are stable, their motion during a series of standardized facial animations was subtracted from that of the facial and headcap landmarks to estimate their movement within the face. This residual movement was used to determine which points are relatively stable (< or = 1.5 mm of movement) and which are not (> or = 1.5 mm of movement). Headcap landmarks were found to be suitable as references during smile, cheek puff, and lip purse animations, and during talking. In contrast, skin-based landmarks were unsuitable as references because of their considerable and highly variable movement during facial animation. Second, the facial movements of patients with obvious facial deformities were compared with those of matched controls to characterize the face validity of three-dimensional tracking. In all instances, pictures that appear to be characteristic of the various functional deficits emerged. CONCLUSIONS: Our results argue that tracking instrumentation is a potentially useful tool in the measurement of facial mobility.

Technique for making flexible impression trays for the microstomic patient.

This impression technique can be used for patients in whom routine use of stock impression trays is hindered by microstomia. Putty wash material can be manipulated with minimal effort and time. Placing the completed preliminary impression in a free-flowing mix of dental stone stabilizes the impression material and facilitates boxing and pouring of the impression. The resultant preliminary casts can then be used for diagnostic purposes and for making rigid sectional trays for final impressions.

Fabrication of a flexible prosthesis for the edentulous scleroderma patient with microstomia.

The exercise prosthesis serves as an interim device to achieve an increased opening of the jaws. Once the patient is able to insert the existing denture or when an impression tray can be placed in the patient's mouth without extreme discomfort, its purpose will have been achieved; the dentures should be relined or remade. Some patients need only achieve slight increases in the maximal vertical oral opening to make prosthetic treatment possible (Fig. 6). While this technique is useful for the edentulous scleroderma patient, it may have application for individuals with limited mouth opening secondary to facial burns or other scarring. It is suggested as an alternative to surgical correction through bilateral commissurotomies.

A study of gustatory and olfactory function in patients with craniofacial anomalies.

Olfactory and gustatory function can be reiably studied in patients with craniofacial anomalies over the age of 7 years. In our unoperated patients with orbital hypertelorism or craniofacial dysostosis, preoperative evaluation of the olfactory and gustatory functions showed normal values. The same techniques were employed to study any changes in these modalities following reconstructive craniofacial surgery, and the results are presented.