# Simultaneous closure of a perilymphatic fistula and placement of cochlear implant in a case of complex inner ear malformation

## TL;DR

A child with inner ear malformation and a perilymphatic fistula underwent successful surgery to close the fistula and receive a cochlear implant, improving long-term hearing and development.

## Contribution

Demonstrates the successful simultaneous surgical closure of a perilymphatic fistula and cochlear implant placement in a complex pediatric case.

## Key findings

- Surgical closure of a perilymphatic fistula and cochlear implant placement improved hearing outcomes in an 8-month-old child.
- The child showed good hearing results with a cochlear implant and only moderate speech delay at age 6.
- Early intervention with cochlear implants and fistula closure can maximize developmental potential in cases of inner ear malformations.

## Abstract

In young infants, under the age of one‐year, cochlear malformation with profound hearing loss complicated by a perilymphatic fistula (PLF), presents a serious clinical challenge, warranting immediate audiological and surgical intervention. Timely PLF detection and closure, along with an early CI can significantly improve the prognosis of such patients and helps them in achieving their maximum hearing and developmental potential, in the long term.

Inner ear malformation (IEM) with incomplete partition and cystic cochlea is mostly accompanied by profound hearing loss. It gets further complicated with other malformations such as a perilymphatic fistula (PLF). This case concerns an 8‐month‐old child cochlear malformation and profound hearing loss. Surgical intervention identified a PLF at the stapedial footplate, which was successfully closed. The surgery also included the placement of a cochlear implant (CI) in the right ear, via the round window. The left ear was equipped with hearing aids, with persistent hearing thresholds at 70–80 db. At the age of 6 years, the child showed a good hearing outcome with the CI, with only moderate speech delay. Cochlear malformation accompanied by a perilymphatic leakage warrants immediate surgical closure of the PLF, to minimize the risk of bacterial meningitis. Wherever possible, the feasibility of a CI should be explored in such cases and a CI should be placed for treatment of hearing loss. Audiological and speech outcomes may vary with the use of the CI, especially in cases of IEM. However, an early CI coupled with timely PLF detection and closure can help children with profound hearing loss, in achieving their maximum hearing and developmental potential, in the long run.