THE DEAFNESS, PREAURICULAR SINUS, EXTERNAL EAR ANOMALY AND COMMISSURAL LIP PITS SYNDROME - OTOLOGIC, VESTIBULAR AND RADIOLOGICAL FINDINGS

Commissural lip pits, pinna dysplasia, pre-auricular sinus and hearing loss constitute a recently described autosomal dominant branchial arc h syndrome. In a large family, eight out of the 74 members were also a ffected by conductive hearing loss. No inner ear abnormalities could b e demonstrated on the CT scans. In three patients (four ears) out of f our patients (six ears), exploratory tympanotomy revealed serious ossi cular chain anomalies. In one ear, round window aplasia was also prese nt. Long-term hearing improvement could only be achieved in one ear.