Effectiveness of cochlear implant in inner ear bone malformations with anterior labyrinth involvement

Vera, Juan Miguel Palomeque; Gómez-Hervás, Javier; Fernández-Prada, María; Alba-Saida, García Negro; Ramírez, Amanda Rocío González; Quevedo, Manuel Sainz

doi:10.1016/j.ijporl.2014.12.029

Cited by 4 publications

(1 citation statement)

References 27 publications

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“…No other mastoid anomalies were reported in the implanted patients; however, extensive cochlear, vestibular, and ossicular malformations were observed alongside deviation of the facial nerve. In a case series conducted by Palomeque Vera et al, all five patients with inner ear malformations (including cochlear hypoplasia), benefitted from CI; however, those with major malformations had worse audiological outcomes 89 . Successful implantation has also been reported in patients with EVA, a feature noted in three patients receiving implants 90 .…”

Section: Discussionmentioning

confidence: 97%

Anatomical and audiological considerations in branchiootorenal syndrome: A systematic review

Biggs

Crundwell

Metcalfe

et al. 2022

Laryngoscope Investig Oto

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Objective Establish anatomical considerations, audiological outcomes, and optimal management in patients with branchiootic/branchiootorenal syndrome (BO/BOR). Methods Databases reviewed: Medline, Pubmed, Embase, Web of Science, Cochrane Collection, and http://clinicaltrials.gov. Clinical or radiological studies of patients with BOR syndrome describing either the audiological profile or anatomical changes were included. Articles in which BOR syndrome was associated with other syndromes, and those that were focused only on general and genetic aspects of BOR syndrome were excluded. Articles were assessed using Oxford Centre for Evidence‐Based Medicine (OCEBM) grading system and the Brazzelli risk of bias tool for nonrandomized studies. Results Searches identified 379 articles. Of these, 64 studies met the inclusion criteria, reporting outcomes in 482 patients from at least 95 families. In 308 patients, hearing loss was categorized as sensorineural (29%), conductive (20%), and mixed (51%). Hearing outcomes were variable in terms of onset, pattern, and severity; ranging from mild to profound deafness. One hundred sixty‐nine patients presented with inner ear anomalies, 145 had middle, and 151 had external ear abnormalities. In 44 studies, 58 ear operations were described. Mixed outcomes were reported in patients managed with hearing aids or middle ear surgery; however, successful cochlear implantation was described in all five cases. Conclusion The anatomical and audiological profiles of patients with BO/BOR are variable. A range of surgical procedures were described, however lacked objective outcome measures. Given the range of anatomical variants, management decisions should be made on an individual basis including full audiological and radiological assessment. Level of evidence NA.

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Section: Discussionmentioning

confidence: 97%