Content area
Full Text
Background
Otosclerosis in childhood and adolescence or juvenile otosclerosis is a rare clinical entity. In adults, otosclerosis is a well-known cause of conductive hearing loss (CHL) mainly affecting patients aged between 15 and 45 years. However, microscopic foci of otosclerosis have been found in 0.6% of autopsies of individuals less than 5 years of age. 1
With regard to the genetic background, juvenile otosclerosis is a multifactorial disease, associated with autosomal dominant inheritance with incomplete penetrancy of approximately 40-45%. 2 In adults, stapedectomy is the treatment of choice, in which the stapes is surgically removed and replaced with a prosthesis. In children, stapedectomy remains a challenging procedure and a ground of dispute with questions about the indicated age of operation, the operating technique and the management of possible complications. Alternative conservative treatment options are sound amplification with a hearing aid, a bone anchored hearing aid or a 'watch-and-wait' strategy.
The aim of this study is to report on a child suffering from juvenile otosclerosis, obscured by secretory otitis media, and to review the literature on this topic.
Case presentation
A 9-year-old boy presented to our clinic, suffering from moderate hearing loss. According to his parents, the hearing loss had progressively deteriorated over the past 2 years, while he had fully developed normal speech. No family history of hearing loss was reported. The clinical examination revealed bilateral secretory otitis media and tympanometry revealed a type B pattern. Pure tone audiogram showed CHL with air-bone gap of 45 dB in the right ear and 22 dB in the left ear ( figure 1 A). The patient underwent examination of the ears under general anaesthesia, bilateral myringotomy and grommet insertion, during which a significant amount of glue was aspirated; a small section of the adenoids was also removed at the same time. Two months later, the child still reported of hearing loss and pure tone audiogram demonstrated slight improvement of only 10 dB of the ABG in all frequencies ( figure 1 B). High-resolution CT scan did not show any otosclerotic foci around the stapes footplate, and no congenital dysplasia of the external and middle ear.
Differential diagnosis
The high degree of hearing loss and the progressive character of the hearing loss led us to suspect that the...