Case Report: Simultaneous repair of cerebrospinal fluid otorrhea and cochlear implantation in a patient with single-sided deafness and inner ear anomaly

By
Sen Chen
Huamao Cheng
March 30, 2026
0 min

Frontiers In Surgery

Overview

A 5-year-old male with unilateral profound sensorineural hearing loss and spontaneous cerebrospinal fluid (CSF) otorrhea due to inner ear malformation underwent successful surgical repair of the CSF leak and concurrent cochlear implantation. Postoperative outcomes showed no recurrence of CSF otorrhea and restoration of hearing in the affected ear.

Background

CSF otorrhea involves leakage of cerebrospinal fluid through the ear, often caused by trauma, congenital malformations, or iatrogenic factors. Spontaneous CSF otorrhea in children is frequently linked to congenital inner ear dysplasia, which can create abnormal communication between the subarachnoid space and middle ear. These malformations often coincide with sensorineural hearing loss, posing challenges in managing both CSF leakage and auditory rehabilitation simultaneously.

Data Highlights

Test	Right Ear	Left Ear
Pure-tone audiometry	Normal hearing	Profound sensorineural hearing loss
Auditory Brainstem Response (ABR)	Normal	Profound hearing loss
Auditory Steady-State Response (ASSR)	Not specified	Thresholds >85 dB nHL
Acoustic Immittance	Type As curve	Type B curve
Postoperative Sound Field Audiometry	Not applicable	Average threshold 31 dB HL

Key Findings

Imaging revealed incomplete partition type I cochlear malformation with a cystic cochlea lacking a modiolus and dilated vestibule on the left side.
CSF otorrhea was localized intraoperatively at the oval window with a fistulous opening at the stapes footplate.
Surgical repair involved sealing the fistula with temporalis fascia and surgical glue, followed by cochlear implantation via the round window niche.
Postoperative imaging confirmed correct electrode placement and no CSF leak recurrence.
Hearing was restored in the left ear with an average threshold of 31 dB HL one month after surgery.

Clinical Implications

This case demonstrates that simultaneous repair of spontaneous CSF otorrhea and cochlear implantation is feasible and effective in pediatric patients with inner ear malformations and unilateral profound hearing loss. Early identification and surgical intervention can prevent life-threatening complications such as meningitis while restoring auditory function. Multidisciplinary evaluation including imaging and audiological testing is critical for optimal surgical planning.

Conclusion

Simultaneous surgical management of CSF otorrhea and cochlear implantation can successfully address both cerebrospinal fluid leakage and profound unilateral hearing loss in pediatric patients with inner ear malformations. This approach offers a promising treatment strategy to restore hearing and prevent complications.

References

Author/Source/Year -- Surgical Management of Cerebrospinal Fluid Otorrhea and Cochlear Implantation in a Pediatric Patient with Unilateral Hearing Loss and Inner Ear Malformation