[Hearing loss in osteogenesis imperfecta--casuistic demonstration].

INTRODUCTION

Osteogenesis imperfecta (OI) is a genetic disorders of connective tissue. Hearing loss has been repeatedly reported as a major syndrome of osteogenesis imperfecta. The hearing loss in OI is predominantly of the conductive type.

MATERIAL AND

METHODS

The family (mother and two daughters) suffering from osteogenesis imperfecta are presented.

RESULTS

Audiological examination is presented. It revealed various types of hearing loss in members of family (conductive, sensorineural, mixed).

CONCLUSIONS

On this example variety of hearing problems in this disease are demonstrated. Etiology and classification of osteogenesis imperfecta, clinical proceeding and treatment are presented.