Two siblings with progressive, fluctuating hearing loss after head trauma, treated with cochlear implantation

The Journal of Laryngology and Otology
M J F de WolfCor W R J Cremers

Abstract

Description of two siblings with unexplained, progressive, perceptive hearing loss after head trauma. Case report. Two siblings aged six and eight years old with bilateral, intermittent but progressive hearing loss. These patients had a c.1172G>A (p.Ser391Asn) mutation in the SLC26A4 gene, which has not previously been reported and which caused Pendred or enlarged vestibular aqueduct syndrome. The diagnosis was based on the perceptive hearing loss, computed tomography findings and mutation analysis. The patients were each fitted with a cochlear implant because of their severe, progressive, perceptive hearing loss with deep fluctuations. The results were good. Further testing for the presence of an enlarged vestibular aqueduct is recommended when children present with sudden progression in perceptive hearing loss, whether or not in combination with head trauma. Cochlear implantation is indicated in patients with persistent, progressive hearing loss that leads to deafness. Implantation can be undertaken successfully despite cochlear hypoplasia.

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Citations

Sep 15, 2012·Hearing Research·Robert W EppsteinerRichard J H Smith
May 15, 2015·European Archives of Oto-rhino-laryngology : Official Journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : Affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery·Juan Miguel Palomeque VeraManuel Sainz Quevedo
Sep 4, 2015·Clinical Otolaryngology : Official Journal of ENT-UK ; Official Journal of Netherlands Society for Oto-Rhino-Laryngology & Cervico-Facial Surgery·J W I van NieropHenricus P M Kunst
Oct 17, 2015·Clinical Otolaryngology : Official Journal of ENT-UK ; Official Journal of Netherlands Society for Oto-Rhino-Laryngology & Cervico-Facial Surgery·A M M OonkRonald J E Pennings
Nov 20, 2014·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Bo Jan NoordmanPaul Merkus
Aug 11, 2017·Otology & Neurotology : Official Publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology·Kyung Jin RohJae Young Choi

Related Concepts

SLC26A11 protein, human
DNA Mutational Analysis
Temporal Region Trauma
Sensory Hearing Loss
Tomography, X-Ray Computerized Axial
Cochlear Implant Procedure
Missense Mutation
Permease
Cochlear Diseases
Cochlear Implants

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