Van der Hoeve Syndrome and Stapes Surgery: Case Reports and a Review – Pages 11-16

C. Mezzedimi1 and D. Passali2

1Otolaryngology Head and Neck Surgery Department, Lucca Hospital, Italy; 2ENT Dept., University of Siena, Italy

DOI: http://dx.doi.org/10.12970/2308-7978.2013.01.01.3

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Abstract: Osteogenesis imperfecta (OI) is a group of genetic disorders that mainly affect the bones. People with this condition have bones that break easily, often from mild trauma or with no apparent cause.

There are at least eight recognized forms of osteogenesis imperfecta, designated type I through type VIII. The classic triad of conductive hearing loss, spontaneous fracture and blue sclera is known as Van der Hoeve Syndrome. The aim of the study is to evaluate some case reports with Van der Hoeve Syndrome treated with stapes surgery and to make a review of this syndrome.

In the period from 2001 to 2009 4 Caucasian patients affected with Van der Hoeve Syndrome underwent stapes surgery. Totally 6 ears were treated.

According to our results, even though the number of patients is limited, stapes surgery in patients with Van der Hoeve syndrome has to be considered a valuable technique in improving hearing if performed by an experienced surgeon.

Stapes surgery is successful in resolving the conductive hearing loss in OI patients, even in the long term. Moreover it could reduce the progression of sensorineural hearing impairment.

The improvement seems to last in time, even though more studies are necessary.

Keywords: Osteogenesis imperfecta, hearing loss, otosclerosis, blue sclera, stapes surgery. Read more →