Simultaneous closure of a perilymphatic fistula and placement of cochlear implant in a case of complex inner ear malformation
Key Clinical Message 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...
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Wiley
2024-09-01
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| Series: | Clinical Case Reports |
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| Online Access: | https://doi.org/10.1002/ccr3.9423 |
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| author | Hanan Almuzaini J. Müller Flatz Wilhelm D. Polterauer M. Schuster |
| author_facet | Hanan Almuzaini J. Müller Flatz Wilhelm D. Polterauer M. Schuster |
| author_sort | Hanan Almuzaini |
| collection | DOAJ |
| description | Key Clinical Message 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. Abstract 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. |
| format | Article |
| id | doaj-art-8c0b088e7eb44d23995d148b182f29bf |
| institution | Kabale University |
| issn | 2050-0904 |
| language | English |
| publishDate | 2024-09-01 |
| publisher | Wiley |
| record_format | Article |
| series | Clinical Case Reports |
| spelling | doaj-art-8c0b088e7eb44d23995d148b182f29bf2024-12-12T10:13:46ZengWileyClinical Case Reports2050-09042024-09-01129n/an/a10.1002/ccr3.9423Simultaneous closure of a perilymphatic fistula and placement of cochlear implant in a case of complex inner ear malformationHanan Almuzaini0J. Müller1Flatz Wilhelm2D. Polterauer3M. Schuster4General and Specialized Surgery Department Medicine College, Taibah University Madinah, KS Saudi ArabiaDepartment of Otorhinolaryngology, Head and Neck Surgery Ludwig‐Maximilians‐University of Munich Munich GermanyDepartment of Radiology Ludwig‐Maximilians‐University of Munich Munich GermanyDepartment of Otorhinolaryngology, Head and Neck Surgery Ludwig‐Maximilians‐University of Munich Munich GermanyDepartment of Otorhinolaryngology, Head and Neck Surgery Ludwig‐Maximilians‐University of Munich Munich GermanyKey Clinical Message 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. Abstract 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.https://doi.org/10.1002/ccr3.9423cochlea implantationdevelopmental delayinner ear malformationmeningitisperilymphatic fistula |
| spellingShingle | Hanan Almuzaini J. Müller Flatz Wilhelm D. Polterauer M. Schuster Simultaneous closure of a perilymphatic fistula and placement of cochlear implant in a case of complex inner ear malformation Clinical Case Reports cochlea implantation developmental delay inner ear malformation meningitis perilymphatic fistula |
| title | Simultaneous closure of a perilymphatic fistula and placement of cochlear implant in a case of complex inner ear malformation |
| title_full | Simultaneous closure of a perilymphatic fistula and placement of cochlear implant in a case of complex inner ear malformation |
| title_fullStr | Simultaneous closure of a perilymphatic fistula and placement of cochlear implant in a case of complex inner ear malformation |
| title_full_unstemmed | Simultaneous closure of a perilymphatic fistula and placement of cochlear implant in a case of complex inner ear malformation |
| title_short | Simultaneous closure of a perilymphatic fistula and placement of cochlear implant in a case of complex inner ear malformation |
| title_sort | simultaneous closure of a perilymphatic fistula and placement of cochlear implant in a case of complex inner ear malformation |
| topic | cochlea implantation developmental delay inner ear malformation meningitis perilymphatic fistula |
| url | https://doi.org/10.1002/ccr3.9423 |
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