Juvenile otosclerosis and congenital stapes footplate fixation. A systematic review and meta-analysis of surgical outcomes and management

Andrew Daniel; Gideon Budiono; Amshuman Rao; Gary Kk Low; Matthew Peter Ellis; Jennifer Lee

doi:10.1016/j.ijporl.2022.111418

Juvenile otosclerosis and congenital stapes footplate fixation. A systematic review and meta-analysis of surgical outcomes and management

Int J Pediatr Otorhinolaryngol. 2023 Mar:166:111418. doi: 10.1016/j.ijporl.2022.111418. Epub 2022 Dec 30.

Authors

Andrew Daniel¹, Gideon Budiono², Amshuman Rao², Gary Kk Low³, Matthew Peter Ellis⁴, Jennifer Lee⁵

Affiliations

¹ Department of Otolaryngology, Head and Neck Surgery, Nepean Hospital, NSW, Australia. Electronic address: andrew.rms.daniel@gmail.com.
² Department of Otolaryngology, Head and Neck Surgery, Nepean Hospital, NSW, Australia.
³ Research Operations, Nepean Hospital, Nepean Blue Mountain Local Health District, Derby St, Kingswood, NSW, 2750, Australia; Sydney Medical School, Faculty of Medicine and Health, University of Sydney, NSW, Australia.
⁴ Department of Otolaryngology, Head and Neck Surgery, NHS Greater Glasgow and Clyde, Glasgow, United Kingdom; Glasgow Medical School, Glasgow University, Glasgow, United Kingdom.
⁵ Department of Otolaryngology, Head and Neck Surgery, Nepean Hospital, NSW, Australia; Department of Otolaryngology, Head and Neck Surgery, The Children's Hospital in Westmead, NSW, Australia.

PMID: 36709714
DOI: 10.1016/j.ijporl.2022.111418

Abstract

Objective: Juvenile Otosclerosis (JO) and Congenital Stapes Footplate Fixation (CSFF) are rare ossicular chain disorders seen in the paediatric population and present with conductive hearing loss. Ongoing controversy exists regarding the role of surgical intervention in JO and CSFF given the poorer hearing outcomes and complications when compared with surgical intervention for adult otosclerosis. The objective of this study is to assess the published data on the surgical outcomes of JO and CSFF in order to guide clinicians and counsel patients on the various medical options for these disease entities.

Methods: A systematic review of MEDLINE, EMBASE and Cochrane was performed with inclusion criteria of children with JO or CSFF and hearing outcomes following stapes surgery. Studies identified by the search were reviewed and assessed by two independent reviewers in line with the PRISMA guidelines.

Results: 464 articles were initially reviewed and 28 articles met inclusion in the systematic review and meta-analysis. A total of 810 ears (473 and 337 cases of JO and CSFF respectively) underwent stapes surgery. Average age at time of surgery for JO and CSFF was 14.3 and 10.2 years old respectively. The mean pre-operative Air-Bone-Gap (ABG) for JO and CSFF was 31.8 ± 5.2 dB and 39.4 ± 10 dB respectively. Following stapes surgery, the mean post-operative ABG for JO and CSFF was 9.6 ± 6 dB and 19.2 ± 12.5 dB respectively. Surgical success rate (defined as ABG <10 dB) was 81% for JO and 41% for CSFF. Mean ABG gain for JO and CSFF was 24.8 dB (95% CI: 18.6-33.1) and 22.6 dB (95% CI: 18.4-27.8) respectively. The reported number of dead ears was 4/473 (0.8%) for JO and 2/337 (0.6%) for CSFF. 23 cases (2.8%) reported sensorineural hearing loss (SNHL) >10 dB.

Conclusion: CSFF was associated with poorer hearing outcomes compared to JO, however both entities showed similar improvement in ABG post operatively. Counselling patients and their families on the surgical success rates and complications of JO or CSFF is an important part of the decision making process when deciding between a surgical option or conservative measures such as hearing aids.

Keywords: Congenital stapes footplate fixation; Hearing outcomes; Juvenile otosclerosis; Pediatric; Stapes surgery.

Publication types

Meta-Analysis
Systematic Review
Review

MeSH terms

Adult
Child
Ear Ossicles
Hearing Loss, Conductive / surgery
Humans
Otosclerosis* / surgery
Retrospective Studies
Stapes / abnormalities
Stapes Surgery*
Treatment Outcome