Etiologic diagnosis of sensorineural hearing loss in adults.
OBJECTIVE
To determine the etiology of adult-onset sensorineural hearing loss.


METHODS
This is a prospective cohort study of 60 adult subjects with bilateral sensorineural hearing loss of no obvious etiology by medical history and physical examination. These patients were evaluated at an academic medical center and underwent evaluation by high-resolution computed tomography of the temporal bone, autoimmune panel, and DNA testing for mutations of both the GJB2 gene and the mitochondrial DNA (1555A>G and 7445A>G).


RESULTS
An etiologic diagnosis was achieved in 6 patients: cochlear otosclerosis, 1 case; dilated vestibular aqueduct, 1 case; a mitochondrial DNA 7445A>G mutation, 3 cases; and a mitochondrial DNA 1555A>G mutation, 1 case.


CONCLUSIONS
This result underscores the importance of a search for the etiology of a hearing deficit in adult patients. There are specific interventions now available for the management of hearing-impaired patients with cochlear otosclerosis and mitochondrial DNA mutations.
Angeli
Simon I
SI
Department of Otolaryngology, University of Miami School of Medicine, Florida 33101, USA. sangeli@med.miami.edu
Yan
Denise
D
Telischi
Fred
F
Balkany
Thomas J
TJ
Ouyang
Xiao M
XM
Du
Li L
LL
Eshraghi
Adrian
A
Goodwin
Leslie
L
Liu
Xue Z
XZ
D000328:Adult; D000072259:Connexin 26; D017630:Connexins / Q000235:genetics; D004272:DNA, Mitochondrial / Q000235:genetics; D006319:Hearing Loss, Sensorineural / Q000209:etiology* / Q000235:genetics; D006801:Humans; D009154:Mutation; D013701:Temporal Bone / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed
D017630:Connexins; D004272:DNA, Mitochondrial; C000607042:GJB2 protein, human; D000072259:Connexin 26