Post-surgery Rehabilitative Intervention Based on Imitation Therapy and Mouth-Hand Motor Synergies Provides Better Outcomes in Smile Production in Children and Adults With Long Term Facial Paralysis.

Rehabilitation after free gracilis muscle transfer (smile surgery, SS) is crucial for a functional recovery of the smiling skill, mitigating social and psychological problems resulting from facial paralysis. We compared two post-SS rehabilitation treatments: the traditional based on teeth clenching exercises and the FIT-SAT (facial imitation and synergistic activity treatment). FIT-SAT, based on observation/imitation therapy and on hand-mouth motor synergies would facilitate neuronal activity in the facial motor cortex avoiding unwanted contractions of the jaw, implementing muscle control. We measured the smile symmetry on 30 patients, half of whom after SS underwent traditional treatment (control group, CG meanage = 20 ± 9) while the other half FIT-SAT (experimental group, EG meanage= 21 ± 14). We compared pictures of participants while holding two postures: maximum and gentle smile. The former corresponds to the maximal muscle contraction, whereas the latter is strongly linked to the control of muscle strength during voluntary movements. No differences were observed between the two groups in the maximum smile, whereas in the gentle smile the EG obtained a better symmetry than the CG. These results support the efficacy of FIT-SAT in modulating the smile allowing patients to adapt their smile to the various social contexts, aspect which is crucial during reciprocal interactions.

Facial Expression Time Processing in Typical Development and in Patients with Congenital Facial Palsy.

Temporal dynamics of behavior, particularly facial expressions, are fundamental for communication between individuals from very early in development. Facial expression processing has been widely demonstrated to involve embodied simulative processes mediated by the motor system. Such processes may be impaired in patients with congenital facial palsy, including those affected by Moebius syndrome (MBS). The aims of this study were to investigate (a) the role of motor mechanisms in the processing of dynamic facial expression timing by testing patients affected by congenital facial palsy and (b) age-dependent effects on such processing. Accordingly, we recruited 38 typically developing individuals and 15 individuals with MBS, ranging in age from childhood to adulthood. We used a time comparison task where participants were asked to identify which one of two dynamic facial expressions was faster. Results showed that MBS individuals performed worse than controls in correctly estimating the duration of facial expressions. Interestingly, we did not find any performance differences in relation to age. These findings provide further evidence for the involvement of the motor system in processing facial expression duration and suggest that a sensorimotor matching mechanism may contribute to such timing perception from childhood.

A New Neurorehabilitative Postsurgery Intervention for Facial Palsy Based on Smile Observation and Hand-Mouth Motor Synergies.

Objective: To perform a preliminary test of a new rehabilitation treatment (FIT-SAT), based on mirror mechanisms, for gracile muscles after smile surgery. Method: A pre- and postsurgery longitudinal design was adopted to study the efficacy of FIT-SAT. Four patients with bilateral facial nerve paralysis (Moebius syndrome) were included. They underwent two surgeries with free muscle transfers, one year apart from each other. The side of the face first operated on was rehabilitated with the traditional treatment, while the second side was rehabilitated with FIT-SAT. The FIT-SAT treatment includes video clips of an actor performing a unilateral or a bilateral smile to be imitated (FIT condition). In addition to this, while smiling, the participants close their hand in order to exploit the overlapped cortical motor representation of the hand and the mouth, which may facilitate the synergistic activity of the two effectors during the early phases of recruitment of the transplanted muscles (SAT). The treatment was also aimed at avoiding undesired movements such as teeth grinding. Discussion. Results support FIT-SAT as a viable alternative for smile rehabilitation after free muscle transfer. We propose that the treatment potentiates the effect of smile observation by activating the same neural structures responsible for the execution of the smile and therefore by facilitating its production. Closing of the hand induces cortical recruitment of hand motor neurons, recruiting the transplanted muscles, and reducing the risk of associating other unwanted movements such as teeth clenching to the smile movements.

Children with facial paralysis due to Moebius syndrome exhibit reduced autonomic modulation during emotion processing.

BACKGROUND: Facial mimicry is crucial in the recognition of others' emotional state. Thus, the observation of others' facial expressions activates the same neural representation of that affective state in the observer, along with related autonomic and somatic responses. What happens, therefore, when someone cannot mimic others' facial expressions? METHODS: We investigated whether psychophysiological emotional responses to others' facial expressions were impaired in 13 children (9 years) with Moebius syndrome (MBS), an extremely rare neurological disorder (1/250,000 live births) characterized by congenital facial paralysis. We inspected autonomic responses and vagal regulation through facial cutaneous thermal variations and by the computation of respiratory sinus arrhythmia (RSA). These parameters provide measures of emotional arousal and show the autonomic adaptation to others' social cues. Physiological responses in children with MBS were recorded during dynamic facial expression observation and were compared to those of a control group (16 non-affected children, 9 years). RESULTS: There were significant group effects on thermal patterns and RSA, with lower values in children with MBS. We also observed a mild deficit in emotion recognition in these patients. CONCLUSION: Results support "embodied" theory, whereby the congenital inability to produce facial expressions induces alterations in the processing of facial expression of emotions. Such alterations may constitute a risk for emotion dysregulation.

Autonomic Responses to Emotional Stimuli in Children Affected by Facial Palsy: The Case of Moebius Syndrome.

According to embodied simulation theories, others' emotions are recognized by the unconscious mimicking of observed facial expressions, which requires the implicit activation of the motor programs that produce a specific expression. Motor responses performed during the expression of a given emotion are hypothesized to be directly linked to autonomic responses associated with that emotional behavior. We tested this hypothesis in 9 children (M age = 5.66) affected by Moebius syndrome (MBS) and 15 control children (M age = 6.6). MBS is a neurological congenital disorder characterized by underdevelopment of the VI and VII cranial nerves, which results in paralysis of the face. Moebius patients' inability to produce facial expressions impairs their capacity to communicate emotions through the face. We therefore assessed Moebius children's autonomic response to emotional stimuli (video cartoons) by means of functional infrared thermal (fIRT) imaging. Patients showed weaker temperature changes compared to controls, suggesting impaired autonomic activity. They also showed difficulties in recognizing facial emotions from static illustrations. These findings reveal that the impairment of facial movement attenuates the intensity of emotional experience, probably through the diminished activation of autonomic responses associated with emotional stimuli. The current study is the first to investigate emotional responses in MBS children, providing important insights into the role of facial expressions in emotional processing during early development.

A proposal for new neurorehabilitative intervention on Moebius Syndrome patients after 'smile surgery'. Proof of concept based on mirror neuron system properties and hand-mouth synergistic activity.

Studies of the last twenty years on the motor and premotor cortices of primates demonstrated that the motor system is involved in the control and initiation of movements, and in higher cognitive processes, such as action understanding, imitation, and empathy. Mirror neurons are only one example of such theoretical shift. Their properties demonstrate that motor and sensory processing are coupled in the brain. Such knowledge has been also central for designing new neurorehabilitative therapies for patients suffering from brain injuries and consequent motor deficits. Moebius Syndrome patients, for example, are incapable of moving their facial muscles, which are fundamental for affective communication. These patients face an important challenge after having undergone a corrective surgery: reanimating the transplanted muscles to achieve a voluntarily control of smiling. We propose two new complementary rehabilitative approaches on MBS patients based on observation/imitation therapy (Facial Imitation Therapy, FIT) and on hand-mouth motor synergies (Synergistic Activity Therapy, SAT). Preliminary results show that our intervention protocol is a promising approach for neurorehabilitation of patients with facial palsy.

Comparison of speech perception performance between the Nucleus 24 and Nucleus 24 Contour cochlear implant systems.

OBJECTIVE: To compare the 1-, 3-, and 6-month postoperative speech perception scores obtained by a group of subjects who received the new perimodiolar array (Nucleus Contour) cochlear implant with those obtained by a group of subjects implanted with the straight electrodes of the previous-generation Nucleus 24 device. MATERIAL AND METHODS: The speech performance of 10 postlingually deafened adults implanted with the Nucleus Contour device was compared with that of matched controls who received the Nucleus 24 model. Objective measures included word and sentence speech recognition scores. RESULTS: Patients implanted with the Nucleus Contour device obtained significantly higher word and sentence recognition scores after short-term use of the implant compared to those obtained by patients implanted with the Nucleus 24 model. CONCLUSION: Further long-term studies are required to determine whether the Nucleus Contour CI recipients continue to improve over time.

Cochlear implantation and Cogan syndrome.

OBJECTIVE: To evaluate outcomes and issues pertaining to cochlear implantation in a group of subjects affected by Cogan syndrome. STUDY DESIGN: Prospective cohort. SETTING: Department of Ophthalmology and Otorhinolaryngology, University of Parma. PATIENTS: Five postlingually deafened adults suffering from a typical form of Cogan syndrome who underwent cochlear implantation. MAIN OUTCOME MEASURES: Benefit from cochlear implantation as measured by word and everyday sentence recognition tests. Surgical issues and postoperative complications were also evaluated. RESULTS: In two cases, intracochlear electrodes were inserted into the scala vestibuli because of the ossification of the scala tympani. Two patients experienced a recurrence of keratitis the day after surgery. To date, with a follow-up of 1 to 4 years, no patient has experienced flap complications or other local or systemic complications. At the 12-month postoperative evaluation, all patients had gained useful open-set speech perception, achieving a mean score of 91% and 95% on word and everyday sentence recognition tests, respectively. CONCLUSIONS: Patients deafened by Cogan syndrome demonstrated high levels of speech understanding after undergoing cochlear implantation. Obliteration of the cochlea may complicate electrode implantation, requiring modifications of the surgical technique. Stress consequent to the surgical procedure may instigate an acute phase of the basic illness.

Multichannel cochlear implantation in radical mastoidectomy cavities.

OBJECTIVE: We report on our experience in cochlear implantation in patients with radical mastoidectomy cavities. Study Design, Setting, and Methods: Retrospectively, records of patients from the Department of Otolaryngology, University of Parma between December 1991 and March 2000 were reviewed, and 6 postlingually deafened adults who received a cochlear implant in a radical cavity were identified. Speech performances were evaluated in terms of bisyllabic word and sentence recognition and common phrase comprehension. RESULTS: To date, with a follow-up of 1 to 9 years, no patient has experienced extrusion of electrodes or other local or intracranial complications. Mean bisyllabic word and sentence recognition scores were 74% and 80%, respectively. Mean comprehension score for common phrases was 86%. CONCLUSION: By obliterating and isolating the radical mastoidectomy cavity from the outer environment, patients who previously had undergone radical surgery of the middle ear can be safely implanted with satisfactory hearing results.

Comparison of speech perception benefits with SPEAK and ACE coding strategies in pediatric Nucleus CI24M cochlear implant recipients.

Nine congenitally deaf children who received a Nucleus CI24M cochlear implant and who were fitted with the SPrint speech processor participated in this study. All subjects were initially programmed with the SPEAK coding strategy and then converted to the ACE strategy. Speech perception was evaluated before and after conversion to the new coding strategy using word and Common Phrase speech recognition tests in both the presence and absence of noise. In quiet conditions, the mean percent correct scores for words were 68.8% with SPEAK and 91% with ACE; for phrases the percentage was 66.6% with SPEAK and 85.5% with ACE. In the presence of noise (at +10 dB signal-to-noise ratio), the mean percent correct scores for words were 43.3% with SPEAK compared to 84.4% with ACE; for phrases the percentage was 41.1% with SPEAK and 82.2% with ACE. Statistical analysis revealed significant improvement in open-set speech recognition with ACE compared to SPEAK. Preliminary data suggest that converting children from SPEAK to the ACE strategy improves their performance. Subjects showed significant improvements for open-set word and sentence recognition in quiet as well as in noise when ACE was used in comparison with SPEAK. The greatest improvements were obtained when tests were presented in the presence of noise.

Nucleus multichannel cochlear implantation in partially ossified cochleas using the Steenerson procedure.

OBJECTIVE: The purpose of this study was to report hearing results obtained in a group of subjects who received scala vestibuli implantation because of cochlear ossification and to compare these results to those in patients with scala tympani implantation. STUDY DESIGN: Retrospective analysis of consecutive cochlear implant procedures. SETTING: Department of Otolaryngology, University of Parma. PATIENTS: Five postlingually deafened adults with an electrode array placed into the scala vestibuli were compared with the speech performance of matched controls who had the electrode array inserted into the scala tympani. MAIN OUTCOME MEASURES: Measures included vowel and consonant speech identification scores, bisyllabic word and sentence speech recognition scores, and common phrases comprehension scores. RESULTS: No significant difference was detected on speech performances between the subjects with scala vestibuli implantation and the control group. CONCLUSION: Scala vestibuli implantation appears to be an excellent alternative in cases in which scala tympani is found to be not patent.