Update on congenital stapes footplate fixation and juvenile otosclerosis

This paper aims to contribute to improved diagnostic accuracy, review outcomes of surgery, and provide guidance on how patients and caregivers are counselled about treatment options for juvenile otosclerosis (JO) and congenital stapes footplate fixation (CSFF).

In JO, there is abnormal bone resorption and recalcification leading to progressive conductive or mixed hearing loss depending on the location of affected bone. There is a higher rate of obliterative otosclerosis in children compared to adults and thus consideration should be given to proceed with earlier surgical intervention, with stapedotomy regarded as a safe option. CSFF occurs due to congenital abnormalities in the annular ligament of the footplate and can be associated with other genetic conditions and syndromes. It is characterized by maximal, nonprogressive conductive hearing loss. Stapedotomy can be curative for hearing loss, though the rate of success is reported to be less than in JO, so hearing aids and implants may be more appropriate in some cases.

Accurate diagnosis of JO and CSFF is of key importance because outcomes of surgery differ. High resolution computerized tomography of the petrous temporal bones should be performed to confirm diagnosis and to inform risks and benefits of operative intervention. Hearing rehabilitation should be addressed in a timely manner to optimize learning.