Craniofacial microsomia syndrome is a congenital maxillofacial dysostosis characterized by a number of symptoms described by the international classification OMENSplus. Among them are the underdevelopment of the outer, middle and inner ear, denoted by the letter E. Determination of indications for hearing-improving operations in patients with craniofacial microsomia syndrome is possible using the Jahrsdoerfer scale after audiometry and multislice computed tomography.

THE PURPOSE OF THIS STUDY

Is to determine the algorithm for complex multidisciplinary treatment of patients with a symptom of microtia and atresia of the external auditory canal in craniofacial microsomia syndromes with a demonstration of the features based on a clinical example.

MATERIALS AND METHODS

For the period from 2011 to 2022, 125 patients from 0 to 18 years old with maxillofacial dysostoses were selected at the KCCHLPHS Clinic for 184 beds, of which 111 children with craniofacial microsomia syndrome (88.8%). Of the total number of 111 people, 40 examined underwent MSCT (36%).

RESULTS

It was found that the distribution of symptoms according to the degree of underdevelopment of the outer and middle ear was as follows: E0 — 22 people (19.8%), E1 — 11 people (9.9%), E2 — 16 people (14.4%), E3 — 62 people (55.9%). All patients with severe lesions (E3) had ear canal atresia and ossicular hypoplasia. In 10 patients (9%), cochlear hypoplasia was noted. In the case of moderate severity of the lesion (E2), all patients had a narrowing of the external auditory canal, in 8 patients (7%) — moderate hypoplasia of the auditory ossicles. From 2019 to 2022, out of 110 patients examined, 15 aesthetic reconstructions of the auricle using a costal cartilage autograft, 2 hearing-improving surgeries were performed. Of the two hearing —improving operations, an effective result was achieved in one case.

CONCLUSIONS

Patients with a symptom of microtia and atresia of the external auditory canal with craniofacial mircosomia are shown a comprehensive multidisciplinary approach to examination and treatment.