Document Detail

Audiological outcomes after cochlear implantation in a patient with Melnick-Needles syndrome.
MedLine Citation:
PMID:  18568526     Owner:  NLM     Status:  MEDLINE    
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.
Annelies Vermeiren; Andrzej Zarowski; Jean-Pierre Fryns; Filiep Vanpoucke; Thomas Somers; Erwin Offeciers
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Acta oto-laryngologica     Volume:  128     ISSN:  0001-6489     ISO Abbreviation:  Acta Otolaryngol.     Publication Date:  2008 Jul 
Date Detail:
Created Date:  2008-06-23     Completed Date:  2008-11-18     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0370354     Medline TA:  Acta Otolaryngol     Country:  Norway    
Other Details:
Languages:  eng     Pagination:  814-20     Citation Subset:  IM    
University ENT Department, St Augustinus Hospital, Wilrijk, Belgium.
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MeSH Terms
Cochlear Implantation / methods*
Follow-Up Studies
Hearing / physiology*
Magnetic Resonance Imaging
Osteochondrodysplasias / diagnosis,  physiopathology*,  surgery
Tomography, X-Ray Computed

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