Spontaneous cerebrospinal fluid otorrhea from a tegmen defect: transmastoid repair with minicraniotomy

Spontaneous cerebrospinal fluid (CSF) otorrhea is a rare condition that presents in 2 clinical categories. In congenital labyrinthine malformations, it leads to bouts of meningitis in a hearing-impaired child. In the adult age group, a spontaneous CSF leak almost always results from a dural and bony defect in the tegmen area. Possible pathogenic mechanisms include progressive sagging and rupture of dura through a congenital tegmen dehiscence and progressive bone erosion by aberrant arachnoid granulations. These patients usually present with a middle ear effusion, resulting in clear discharge after myringotomy with tube insertion. Based on 4 patients with a CSF leak from a tegmen defect, this report reviews the clinical findings and diagnostic approach. The surgical management by a 5-layer closure using a transmastoid approach with minicraniotomy is outlined. This procedure offers a relatively simple and reliable method for repair without the inherent risks of a middle fossa craniotomy.