A Case of Oculoauriculovertebral Spectrum with Ossicular Deformity Managed by Oval Window Fenestration / 대한이비인후과학회지

ABSTRACT

Oculoauriculovertebral spectrum (OAVS) is a disease which has wide range of phenotypic expression from hemifacial microsomia. Hemifacial microsomia denotes unilateral microtia, mandibular hypoplasia to Goldenhar syndrome, which consists of epibulbar dermoids and vertebral anomalies in addition. Various auricular, external ear and middle ear deformities are combined in this disease spectrum. We experienced a case which was characterized as OAVS. The patient complained about congenital hearing loss and showed unilateral conductive hearing loss. Microtia type I with mild stenosis of ear canal, hemifacial microsomia and abnormality of cervical vertebrae were found. Exploration revealed middle ear anomalies with ossicular deformity and aberrant facial nerve. To correct the conductive hearing loss, oval window fenestration was performed with KTP laser and piston wire prosthesis was inserted. Hearing was improved successfully after the surgery. It should be stressed that hearing should be carefully assessed in the patients with OAVS, regardless of the type of clinical findings. The correction of anomalies of the middle ear would be one of the important points for otolaryngologists in treatment of OAVS.