Good speech recognition and quality-of-life scores after cochlear implantation in patients with DFNA9.
OBJECTIVE
To compare audiometric and quality-of-life results in DFNA 9 patients who received a cochlear implant with cochlear implant patients with adult-onset progressive sensorineural hearing loss.


METHODS
Prospective comparative design; results were collected cross-sectionally.


METHODS
Tertiary referral center.


METHODS
Eleven DFNA 9 patients were included in the study as well as a comparative group of 39 post-lingually deafened cochlear implant subjects with adult-onset progressive sensorineural hearing loss.


METHODS
All patients received a cochlear implant. Subjects were implanted with either the Nucleus 24 M/RCS or Med-el Combi 40+ cochlear implant systems implementing the SPEAK, ACE, or CIS+ coding strategies.


METHODS
Speech recognition was determined by means of phonetically balanced monosyllabic word lists. The Hearing Handicap Inventory for Adults, the Glasgow Benefit Inventory, and the Scale for the Prediction of Hearing Disability in Sensorineural Hearing Loss were used to quantify the quality of life.


RESULTS
The results show that the speech perception and the quality of life of the DFNA 9 patients do not differ significantly from the control group (p=0.179; p=0.56).


CONCLUSIONS
In spite of the fact that DFNA 9 is a disease that is known to involve cochlear dendrites, cochlear implantation is a good option for treatment of deafness in DFNA 9.
Vermeire
K
K
University Department of Otorhinolaryngology and Head and Neck Surgery, University Hospital Antwerp, Belgium. katrien.vermiere@uza.be
Brokx
J P L
JP
Wuyts
F L
FL
Cochet
E
E
Hofkens
A
A
De Bodt
M
M
Van de Heyning
P H
PH
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001309:Auditory Threshold; D016022:Case-Control Studies; D003054:Cochlear Implants; D003430:Cross-Sectional Studies; D016326:Extracellular Matrix Proteins; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000150:complications / Q000235:genetics* / Q000534:rehabilitation*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D011506:Proteins / Q000235:genetics*; D011788:Quality of Life; D013067:Speech Perception; D013069:Speech Reception Threshold Test; D016896:Treatment Outcome; D015837:Vestibular Diseases / Q000150:complications / Q000235:genetics
C110668:COCH protein, human; D016326:Extracellular Matrix Proteins; D011506:Proteins