Publication
Title
Audiological outcomes after cochlear implantation in a patient with Melnick-Needles syndrome
Author
Abstract
This is the first report on a successful cochlear implantation in a patient suffering from the Melnick-Needles syndrome (MNS). This case study presents an MNS patient with peri-lingual profound hearing loss, implanted at the age of 38. MNS is a rare X-linked genetic bony dysplasia characterized by skeletal and facial abnormalities. Various soft tissue defects and hearing loss have also been described in MNS; however, this is the first report on bilateral inner ear malformation as a phenotypic feature of MNS. At 15 months after cochlear implantation there was 91% speech identification in open-set monosyllabic CVC test. In noise, the results obtained with the CVC lists were the following: 67% for SNR +15 dB, 52% for SNR +10 dB and 36% for SNR +5 dB. The patient is able to discriminate the differences in all 22 phoneme pairs in the APE phoneme discrimination test. This allows the patient to obtain significant communication ability through the telephone. Conclusions are as follows. 1) Bilateral inner ear malformations and bilateral profound perceptive hearing loss possibly belong to the phenotypic features of MNS. 2) Cochlear implant is potentially a good functional solution for patients suffering from MNS and profound hearing loss. 3) Exceptionally good results on auditory performance have been obtained in this MNS patient with peri-lingual profound hearing loss and almost 36 years duration of deafness.
Language
English
Source (journal)
Acta oto-laryngologica. - Stockholm, 1918, currens
Publication
Stockholm : 2008
ISSN
0001-6489 [print]
1651-2251 [online]
Volume/pages
128:7(2008), p. 814-820
ISI
000256753600016
Full text (Publisher's DOI)
UAntwerpen
Faculty/Department
Research group
Publication type
Subject
Affiliation
Publications with a UAntwerp address
External links
Web of Science
Record
Identification
Creation 06.05.2009
Last edited 12.08.2017
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