Mok, Florence1; Papsin, Blake1; James, Adrian1; Isaacson, Brandon2
1 Department of Otolaryngology – Head & Neck Surgery, Hospital for Sick Chilren, University of Toronto, Canada
2 Department of Otolaryngology – Head & Neck Surgery, University of Texas Southwestern, Dallas, USA
Cochleocele is a rare congenital anomaly typically associated with cochlear hypoplasia in which a pouch of endosteum protrudes through a dehiscent stapes footplate into the middle ear. Rupture is associated with CSF leakage and the risk of meningitis. We review our experience of repair with transcanal endoscopic ear surgery (TEES) and prevention of post-operative meningitis.
– Patients: Five consecutive children at two academic centers, median age at surgery of 16 months (13 – 96 months), with congenital footplate defect, four having CSF leak and one a cochleocele. All had ipsilateral profound hearing loss from cochlear hypoplasia (incomplete partition type 1). Two were identified after developing bacterial meningitis.
– Intervention: TEES repair of stapes footplate with autogenous grafts and Tisseal.
– Main Outcome Measures: Retrospective review of medical records to assess the success of repair and complications. Risk factors for post-operative meningitis were evaluated.
All were successfully repaired with no evidence of post-operative CSF leak (follow up (3 – 54 months). The first patient from each center developed pseudomonas meningitis immediately after surgery which resolved with antibiotics. Changes in preparation for surgery resulted in no further cases of post-operative meningitis. No other complications occurred.
TEES provides a minimally invasive technique for effective closure of perilymph leak from congenital footplate dehiscence even in very young children. Post-operative meningitis may be caused by contamination of the graft with pseudomonas from the ear canal. Sterilization of the ear canal after thorough debridement and anti-pseudomonas antibiotic prophylaxis are recommended.