Abstract
In 18 patients with Usher's syndrome, progressive hearing loss was verified audiologically in eight cases. Despite poor auditory threshold values and low speech discrimination scores, there was only one patient who could not communicate with speech. The possibility of hearing impairment being mainly progressive in Usher's syndrome is discussed.
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References
1.
Regenbogen
L
Coscas
G
. Usher syndrome . In: Oculo-auditory syndromes . New York : Masson Publishing , 1985 : 290 –5 .
2.
Vernon
M
. Usher's syndrome — Deafness and progressive blindness: Clinical cases, prevention, theory, and literature survey . J Chronic Dis 1969 ; 22 : 133 –51 .
3.
Gorlin
RJ
Tilsner
TJ
Feinstein
S
Duvall
AJ
. Usher's syndrome type III . Arch Otolaryngol 1979 ; 105 : 353 –4 .
4.
Kumar
A
Fishman
G
Torok
N
. Vestibular and auditory function in Usher's syndrome . Ann Otol Rhinol Laryngol 1984 ; 93 : 600 –8 .
5.
McLeod
AC
McConnell
FE
Sweeney
A
Cooper
MC
Nance
WE
. Clinical variation in Usher's syndrome . Arch Otolaryngol 1971 ; 94 : 321 –34 .
6.
Belal
A
Jr.
. Usher's syndrome (retinitis pigmentosa and deafness). A temporal bone report . J Laryngol Otol 1975 ; 89 : 175 –81 .
7.
Abraham
FA
Cohen
D
Sohmer
H
. Usher's syndrome: Electrophysiological tests of the visual and auditory systems . Doc Ophthalmol 1977 ; 44 : 435 –44 .
8.
Proctor
C
. Diagnosis, prevention and treatment of hereditary sensorineural hearing loss . Laryngoscope 1977 ; 87 (suppl 7 ).
9.
Davenport
SL
Omen
GS
. The heterogeneity of Usher's syndrome . Presented at the Fifth International Conference on Birth Defects, Montreal, Aug 21–27, 1977 .
10.
Nuutila
A
. Dystrophia retinae pigmentosa-dysacusis syndrome (DRD): A study of the Usher or Hallgren syndrome . J Genet Hum 1970 ; 18 : 57 –88 .
11.
Palva
T
. Finnish speech audiometry . Acta Otolaryngol [Suppl] (Stockh) 1952 (suppl 101 ).
12.
Jauhiainen
T
. An experimental study of the auditory perception of isolated bi-syllable Finnish words [Dissertation] . University of Helsinki , 1974 .
13.
Karjalainen
S
Teräsvirta
M
Kärjä
J
Kääriäinen
H
. An unusual otological manifestion of Usher's syndrome in four siblings . Clin Genet 1983 ; 24 : 273 –9 .
14.
Beatty
CW
McDonald
TJ
Colvard
DM
. Usher's syndrome with unusual otologic manifestation . Mayo Clin Proc 1979 ; 54 : 543 –6 .
15.
Merin
S
Abraham
FA
Auerbach
E
. Usher's and Hallgren's syndromes . Acta Genet Med Gemellol (Roma) 1974 ; 23 : 49 –55 .
16.
Karp
A
Santore
F
. Retinitis pigmentosa and progressive hearing loss . J Speech Hear Disord 1983 ; 48 : 308 –14 .