Congenital spontaneous otogenic cerebrospinal fluid leak as a cause of recurrent meningitis in a 9-year-old child – case report and literature review

Abstract

<b>Introduction:</b> Congenital otogenic CSF leak is a rare cause of meningitis. The underlying reason may be a defect of the fundus of internal auditory meatus with pathological communication with the inner ear and CSF leak to middle ear space. This condition requires surgical closure. </br></br> <b>Case report:</b> A 9-year-old boy with a history of recurrent meningitis – 3 episodes. During the last episode, β-2 transferrin test from ear fluid was positive. The patient complained of leakage of clear fluid through the nose on the right side. Otoscopy revealed a bulging tympanic membrane on the right side. The right ear was deaf with normal hearing on the left side. HRCT revealed congenital deformity of the right inner ear. Right side lateral petrosectomy with blind sac closure of the right external auditory canal was performed. During surgery there was an extensive CSF leak through the oval window niche due to a lack of the stapes footplate in the anterior part. Recovery was uneventful. After 24 months of follow up, no more leakage through the nose was observed. Control magnetic resonance performed one year after surgery showed no CSF in the mastoid cavity. </br></br> <b>Discussion:</b> Inner ear malformations are a rare cause of meningitis, and the most common sites of congenital otogenic CSF leakage include the oval and round window. Surgical closure is the only solution in such cases. Several methods of sealing the inner ear space were described in the literature. Vestibular packing and stapedectomy were most frequently used. However, recurrences were stated in up to 1/2 of cases. Such a risk can be limited with lateral petrosectomy. For this reason, LP can be considered as the treatment of choice in congenital spontaneous otogenic cerebrospinal fluid leak. </br></br> <b>Conclusion:</b> Every case of recurrent meningitis in children should be investigated with a suspicion of congenital ear malformation with spontaneous CSF leakage. Lateral petrosectomy is a safe and effective procedure for cases of otogenic CSF leak with a low risk of recurrence.