Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2021 Sep;73(3):392-394.
doi: 10.1007/s12070-020-02331-9. Epub 2021 Jan 21.

Cochlear Implantation in Pierre Robin Syndrome

Shubhangi Gupta  1 , Mukesh Dagur  2 , Mohnish Grover  1 , Sunil Samdani  1 , Rekha Singh  3 , Rashmi Goyal  2 , Gaurav Mehta  4 , Raghav Mehta  2
Affiliations

Cochlear Implantation in Pierre Robin Syndrome

Shubhangi Gupta et al. Indian J Otolaryngol Head Neck Surg. 2021 Sep.

Abstract

Hereditary hearing loss accounts for nearly 60% of deafness in developed countries and about 30% of them are syndromic. Pierre Robin Syndrome is one such condition. The patient with this syndrome usually presnts with triad of micrognathia, glossoptosis and cleft palate. Hearing loss is mostly conductive but there can be sensorineural hearing loss also. Here we present a case of Pierre Robin Syndrome who presented with congenital hearing loss. He also had bilateral serous otitis media. He underwent cochlear implant surgery and was prescribed antihistaminics and steroid spray for middle ear effusion. Therefore, proper clinical evaluation is required.

Keywords: Cochlear implantation; Congenital hearing loss; Middle ear effusion; Pierre robin syndrome.

PubMed Disclaimer

Figures

Fig. 1
Fig. 1
High resolution computed tomography of temporal bone showing serous otitis media in the right ear (arrow)
Fig. 2
Fig. 2
Edematous middle ear mucosa(*) with cochlear implant placed via posterior tympanotomy (arrow)
See this image and copyright information in PMC

References

    1. Barlow-Stewart K, Mona Sale (2007) Centre for genetics education. Deafness and hearing loss–genetic aspects. The Australasian genetics Resource Book.
    1. Berrettini S (2008) Linee guida per la conduzione dello screening audiologico neonatale nella regione Toscana http://www.fimp.org
    1. Evans KN, Sie KC, Hopper RA, Glass RP, Hing AV, Cunningham ML. Robin sequence: from diagnosis to development of an effective management plan. Pediatr. 2011;127(5):936–948. doi: 10.1542/peds.2010-2615. - DOI - PMC - PubMed
    1. Breugem CC, Evans KN, Poets CF, Suri S, Picard A, Filip C, et al. Best practices for the diagnosis and evaluation of infants with robin sequence: a clinical consensus report. JAMA Pediatr. 2016;170:894–902. doi: 10.1001/jamapediatrics.2016.0796. - DOI - PubMed
    1. Handzić J, Bagatin M, Subotić R, Cuk V. Hearing levels in pierre robin syndrome. Cleft Palate Craniofac J. 1995;32(1):30–36. doi: 10.1597/1545-1569_1995_032_0030_hliprs_2.3.co_2. - DOI - PubMed

LinkOut - more resources