Congenital perilymph fistula causing recurrent meningitis: lessons learnt from a single-institution case series.

OBJECTIVE

To study the steps involved in definitive evaluation and successful management of patients with congenital perilymph fistula presenting with recurrent meningitis.

STUDY

DESIGN

Case series with chart review.

SETTING

Tertiary care center.

SUBJECTS AND

METHODS

The case records of 11 patients (12 ears) treated for congenital perilymph fistula presenting with recurrent meningitis were reviewed to ascertain their clinical, radiological, and intraoperative features and outcome following surgery.

RESULTS

Most patients presented after at least 3 episodes of meningitis (range, 2-10 episodes). Ipsilateral hearing loss was present in 9 of 12 ears, with normal hearing in 3 patients. High-resolution computed tomography and/or magnetic resonance imaging scanning of the temporal bone contributed to the diagnosis in 75% of cases but was normal in 3 cases (25%). Oval window and round window defects were the most common (66.7% and 63.6%, respectively). Four ears (33.3%) had more than 1 defect. The unusual presentations included 2 patients who presented in adulthood, a patient with a defect in the medial wall of the attic, and 3 patients with normal radiological findings. Follow-up ranged from 1 to 11 years (median, 2 years). There were 2 failures following simple fistula closure with cessation of symptoms after vestibular obliteration. No patient was readmitted with recurrent meningitis after definitive surgery.

CONCLUSION

Up to 25% of patients with recurrent meningitis secondary to congenital perilymph fistula may have normal audiological and radiological assessment necessitating exploratory tympanotomy. Vestibular obliteration, rather than simple fistula closure, prevents recurrence.