Patients with Pendred syndrome: is cochlear implantation beneficial?

Clinical Otolaryngology : Official Journal of ENT-UK ; Official Journal of Netherlands Society for Oto-Rhino-Laryngology & Cervico-Facial Surgery
J W I van NieropH P M Kunst

Abstract

To evaluate the benefit of cochlear implantation in patients with Pendred syndrome. Retrospective study. Tertiary centre. Speech perception was measured using a phonetically balanced word list at a sound pressure level of 65 dB. Post-operative phoneme scores at 12-month for adults and 36-month for children with Pendred syndrome were compared to scores of patients with an enlarged vestibular aqueduct (EVA) and a reference group with an unknown cause of hearing impairment. Quality of life was measured with the Nijmegen Cochlear Implant Questionnaire to evaluate the differences between pre- and post-implantation. The mean post-operative phoneme scores were as follows: in the Pendred group, 91% (n = 16; SD = 10) for children and 78% (n = 7; SD = 14) for adults; in the reference group, 79% (n = 59; SD = 20) for children and 73% (n = 193; SD = 18) for adults; and in the EVA group, 84% (n = 6; SD = 7) for children and 66% (n = 12; SD = 22) for adults. A significant difference in speech perception was found between the children of the Pendred group and the reference group of 11.4% (SE = 5.2; P = 0.031). Between the adults, a difference of 11.2% (SE = 6.7; P = 0.094) was found. The difference between the Pendred group and the EVA group ...Continue Reading

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Citations

Jul 12, 2017·Current Opinion in Otolaryngology & Head and Neck Surgery·Geoffrey Casazza, Jeremy D Meier
Feb 14, 2021·Journal of Vestibular Research : Equilibrium & Orientation·François SimonMathieu Beraneck
May 25, 2021·American Journal of Otolaryngology·Taylor E PattersonJeffrey D Carron

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