| Audiological outcomes after cochlear implantation in a patient with Melnick-Needles syndrome. | |
| | |
MedLine Citation:
|
PMID: 18568526 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
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. |
| | |
Authors:
|
Annelies Vermeiren; Andrzej Zarowski; Jean-Pierre Fryns; Filiep Vanpoucke; Thomas Somers; Erwin Offeciers |
Related Documents
:
|
11667986 - Audiological findings in patients with myoclonic epilepsy associated with ragged-red fi... 6881306 - Acoustic neuromas. progression of hearing impairment and function of the eighth cranial... 6443616 - Vertical nystagmus in infants with congenital ocular abnormalities. 7336426 - The inner ear dysfunction in hemodialysis patients. 9648196 - Factors associated with contractures in acute spinal cord injury. 21797896 - Clinical history in the diagnosis of the cardiac syncope-the predictive scoring system. |
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 |
Affiliation:
|
University ENT Department, St Augustinus Hospital, Wilrijk, Belgium. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Adult Audiometry Cochlear Implantation / methods* Female Follow-Up Studies Hearing / physiology* Humans Magnetic Resonance Imaging Osteochondrodysplasias / diagnosis, physiopathology*, surgery Tomography, X-Ray Computed |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Impact of MDM2 single nucleotide polymorphism on tumor onset in head and neck squamous cell carcinom...
Next Document: Cochlear implantation in complete remission in a patient with leukemia.