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Published on: February 29, 2020
Rare Cases of Cerebrospinal Fluid Oto-Rhinorrhoea in Patients with Congenital Inner Ear Malformations
Priyanshi Agarwal1, W V B S Ramalingam1, A V Ramesh1
1Department of ENT and Head and Neck Surgery, BLK Super Speciality Hospital, New Delhi, India.
Insights
Spontaneous cerebrospinal fluid (CSF) leaks from the ear or nose, often linked to inner ear malformations, require prompt diagnosis. Early identification and surgical closure of congenital inner ear defects can prevent serious complications like meningitis and hearing loss.
Area of Science:
- Otolaryngology
- Pediatric Neurology
- Neurosurgery
Background:
- Spontaneous cerebrospinal fluid (CSF) oto-rhinorrhoea is a rare condition, often secondary to congenital inner ear malformations.
- Symptoms include watery discharge from the nose or ear, particularly in head-dependent positions, suggesting a CSF leak.
- Congenital inner ear deformities are associated with meningitis and hearing loss.
Purpose of the Study:
- To highlight the association between congenital inner ear malformations and CSF oto-rhinorrhoea.
- To emphasize the importance of early diagnosis and management of CSF leaks in children.
- To present successful surgical outcomes in pediatric cases.
Main Methods:
- Case report of two children with spontaneous CSF oto-rhinorrhoea.
- Diagnostic evaluation included biochemical analysis of fluid, CT scans of paranasal sinuses and temporal bone.
- Surgical intervention involved closure of the CSF leak from the oval window.
Main Results:
- Case 1: A 1-year-old child with CSF oto-rhinorrhoea, Mondini deformity, and profound right-sided hearing loss.
- Case 2: A 12-year-old male with recurrent meningitis and common cavity malformation of the inner ear, presenting with profound right hearing loss.
- Both patients underwent successful surgical closure of the CSF leak with no recurrence after over a year of follow-up.
Conclusions:
- Congenital inner ear malformations are a significant cause of recurrent meningitis in children.
- High index of suspicion, thorough clinical evaluation, and radiological studies are crucial for diagnosing CSF oto-rhinorrhoea and associated malformations.
- Timely diagnosis and management lead to favorable prognoses and avoidance of severe complications.
Abstract:
Spontaneous cerebrospinal fluid (CSF) oto-rhinorrhoea is rare and may develop secondary to inner ear malformation. Any child discharging watery fluid through nose or ear spontaneously in a head dependent position should be a high index of suspicion of CSF leak. If watery rhinorrhoea is present then apart from biochemical analysis of fluid discharge to confirm it to be CSF, computed tomography of paranasal sinuses and temporal bone should be carried out to differentiate between CSF oto-rhinorrhoea and CSF rhinorrhoea. Congenital deformities of the inner ear can be associated with meningitis and varying degrees of hearing loss. Here we describe two cases, one of CSF oto-rhinorrhea in a 1 year old child who presented with spontaneous watery rhinorrhoea following violent projectile vomiting of 1 month duration. On evaluation, child was found to have CSF oto-rhinorrhoea with right Mondini deformity and profound hearing loss on right side. 2nd case was of 12 years old male with profound hearing loss right ear and recurrent episodes of meningitis diagnosed as common cavity malformation of inner ear. Both children underwent closure of CSF leak from oval window successfully. There was no recurrence after more than 1 year of follow up. Congenital inner ear malformations are an important cause of recurrent meningitis in children and require a high index of suspicion for diagnosis. Thorough clinical evaluation and radiological study is suggested in all cases of CSF oto-rhinorrhoea for the identification inner ear malformation and management of CSF oto-rhinorrhoea. Patients who receive an accurate and early diagnosis can avoid severe complications and have a good prognosis.
