Background and objective.

The Oculo Auriculo Vertebral Spectre (OAVS) refers to a series of malformations in the orbit, mandible, ear, facial nerve as well as spine/vertebral defects. Facial nerve compromise is present in up to 10-45% of the cases. OMENS classification evaluates facial nerve function according to clinical parameters, whereas the eFACE scale system provides a graphic output, it evaluates 3 categories static, dynamic, and synkinetic. Further, eletrophysiological studies are currently one of the most useful and objective methods to evaluate nerve viability and target muscles.

The aim of this investigation is to conduct an electrophysiological characterization and an evaluation with the eFACE scale in patients with facial palsy associated to OAVS.

Methods.

All cases of OAVS associated to facial palsy from our institution were included. Each patient was evaluated with the eFACE scale system. We excluded patients without electrophysiological studies.

Results.

A total of 32 patients were evaluated: 12 male and 20 female, with a mean age of 11.46 years old. Seventeen had a left facial palsy, 14 had right facial palsy and 1 had bilateral involvement. The superior facial nerve branches were affected in 6 patients, 3 had inferior nerve involvement and 23 had involvement of all of the facial nerve branches. According to the eFACE scale, the mean score was 74 (repose) and 56 (dynamic). The peri-ocular score was 72, a mean score of 68 for middle facial third (including smile) and an inferior score of 79. As for the neuroconduction studies, most of them reported a mixed axonopathy, with involvement of 3 branches in 23 patients, 2 branches in 5 and 1 branch in 4 cases. Thirteen patients had involvement of the masseteric nerve branch and 4 in the hypoglossal nerve. As for the electromyography, 5 patients had no activity in the upper third, 3 in the smile muscles and 7 in the depressors.

Conclusions.

The eFACE scale and electrophysiological characterization of patients with facial palsy associated to OAVS provides useful information regarding the facial nerve's branches involvement and the current state of muscular function, both of utmost importance when clinical evaluation is performed before any type of surgical procedure is planned in this patients.

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