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Case report
Oval window perilymph fistula in child with recurrent meningitis and unilateral hearing loss
  1. Miane Min Yan Ng1,
  2. Felice D'Arco2,
  3. Raouf Chorbachi3 and
  4. Robert Nash4,5
  1. 1Ear, Nose and Throat Surgery Department, Great Ormond Street Hospital for Children NHS Trust, London, UK
  2. 2Department of Radiology, NIHR GOSH BRC, Great Ormond Street Hospital for Children NHS Trust, London, UK
  3. 3Department of Audiological Medicine/Audiology/Cochlear Implants, North Thames Cleft Service, Great Ormond Street Hospital for Children NHS Trust, London, UK
  4. 4Department of Paediatric Otolaryngology, Cochlear Implants, Great Ormond Street Hospital for Children NHS Trust, London, UK
  5. 5University College London, London, UK
  1. Correspondence to Dr Miane Min Yan Ng;{at}


A 2-year-old boy presented to Ears, Nose and Throat (ENT) surgeons with unilateral hearing loss. Following a prodrome of upper respiratory tract infection (URTI), he developed two episodes of pneumococcal meningitis in quick succession. This case demonstrates an unusual cause of perilymph fistula diagnosed on imaging and confirmed surgically. He had failed the Newborn Hearing Screening Programme and was therefore referred to audiology, who confirmed profound sensorineural hearing loss in the right ear. MRI showed incomplete partitioning (type 1) of the right cochlea, suggesting cerebrospinal fluid (CSF) leak from the region of the stapes. Exploratory tympanotomy confirmed this, and proceeded to CSF leak repair, obliteration of the Eustachian tube, subtotal petrosectomy, abdominal fat grafting and blind sac closure. Although middle ear effusions are common; particularly in children with recent URTI, the possibility of otogenic CSF leak needs to be considered, especially in cases of recurrent meningitis.

  • ear, nose and throat/otolaryngology
  • radiology
  • congenital disorders

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  • Contributors MMYN, RN took the lead in writing the manuscript with input from all authors. FD'A, RC provided critical feedback, analysis and revision of the manuscript. FD’A contributed to selection and annotation of the images. RN is supervisor of the project.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Parental/guardian consent obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.