Cerebrospinal fluid otorrhea in a patient with congenital inner ear dysplasia

Area of Science:

• Otolaryngology
• Neurosurgery
• Pediatric Surgery

Background:

• Cerebrospinal fluid (CSF) fistulas are rare but serious complications, often associated with congenital anomalies or trauma.
• Congenital inner ear dysplasia can present with various audiovestibular symptoms and may be associated with other developmental abnormalities.
• Surgical intervention is typically required to repair CSF fistulas and prevent complications such as meningitis.

Observation:

• A 10-month-old boy presented with a right-sided CSF fistula and congenital inner ear dysplasia.
• Surgical exploration revealed the fistula's unique location superior to the oval window, with a normally shaped stapes.
• The patient underwent surgical obliteration of the fistula using temporal fascia and fat tissue.

Findings:

• This case represents the first reported instance of a CSF fistula located superior to the oval window.
• Surgical repair involved stapes removal, obliteration of the vestibulum, and closure of the fistula.
• The successful surgical outcome suggests that even rare fistula locations can be effectively managed.

Implications:

• This report expands the understanding of potential CSF fistula locations, particularly in association with congenital inner ear anomalies.
• The surgical technique employed provides a potential approach for managing similar rare fistulas.
• Further temporal bone studies may elucidate the relationship between microfissures and perilymphatic fistulas.