Auditory perception in Alport's syndrome.
Alport's Syndrome is characterized by the presence of renal, hearing and visual disorders.


OBJECTIVE
To characterize the TOAE and the MOES activity (suppression effect) in individuals with Alport's Syndrome.


METHODS
This is a prospective study of a sample included ten individuals with a diagnosis of Alport's Syndrome. MOES recording was made in the presence and absence of contralateral stimulation (CLS) stimulation using the computer software ILO 92 - Otodynamics.


RESULTS
TOAE was present in the global response (A) and in frequency ranges of 1000, 1500, 2000 and 3000 Hz in 4 individuals (40%), and absent in 6 individuals (60%) with hearing loss. We observed no responses at 4000 Hz in the right and left ears. Individuals that presented global responses to TOAE also suppressed that response when there was noise.


CONCLUSIONS
The suppression effect also occurs with TOAE, suggesting that the hearing loss is predominantly the result of cochlear dysfunction.
Viveiros
Carla Mherlyn
CM
Programa de Pós-Graduação dos Distúrbios da Comunicação Humana: Campo Fonoaudiológico, Escola Paulista de Medicina, Universidade Federal de São Paulo, SP.
Pereira
Liliane Desgualdo
LD
Kirsztajn
Gianna Mastroianni
GM
D000293:Adolescent; D000328:Adult; D001301:Audiometry, Pure-Tone; D001308:Auditory Perceptual Disorders / Q000175:diagnosis / Q000209:etiology*; D005072:Evoked Potentials, Auditory / Q000502:physiology; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009394:Nephritis, Hereditary / Q000150:complications / Q000503:physiopathology*; D009847:Olivary Nucleus / Q000502:physiology*; D017084:Otoacoustic Emissions, Spontaneous / Q000502:physiology*; D011446:Prospective Studies