ORIGINAL ARTICLE
ELECTROPHYSIOLOGICAL AND ELECTROACOUSTIC ASSESSMENT OF HEARING IN INDIVIDUALS WITH NEUROFIBROMATOSIS TYPE 1
Raquel Caroline Ferreira Lopes Fontanelli 1, A-G  
,   Nathalia Seppe Fernandes 1, B-C,E-G  
,   Gabriela Ishiguro Silva 1, B-E  
,   Marcelo de Melo Aragão 2, A-B  
,   Ricardo Silva Pinho 2, A-B  
,   Daniela Gil 1, A,D-G  
 
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1
Speech Therapy Department and Audiology, Federal University of São Paulo, Brazil
2
Department of Neurology and Neurosurgery, Support Group for Adolescents and Children with Cancer from Federal University of São Paulo, Brazil
A - Research concept and design; B - Collection and/or assembly of data; C - Data analysis and interpretation; D - Writing the article; E - Critical revision of the article; F - Final approval of article;
CORRESPONDING AUTHOR
Raquel Caroline Ferreira Lopes Fontanelli   

Speech Therapy Department and Audiology, Federal University of São Paulo, Rua Botucatu, 802, 04023-062, São Paulo, Brazil
Publication date: 2021-03-31
 
J Hear Sci 2021;11(1):46–53
 
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ABSTRACT
Background:
Type 1 neurofibromatosis occurs in 1 in every 3,000 individuals, representing 90% of cases of neurofibromatosis. Hearing impairments are not commonly described as an alteration resulting from the pathology; however, sensorineural hearing losses with retrocochlear characteristics may occur as a result of the presence of acoustic neurinomas.

Objectives:
To assess the electrophysiological and electroacoustic hearing characteristics of individuals with neurofibromatosis type 1.

Material and methods:
We assessed 15 patients, 10 females and 5 males, aged between 9 and 31 years, using the following procedures: pure tone audiometry, transient evoked otoacoustic emissions, contralateral suppression of otoacoustic emissions, and brainstem auditory evoked potentials.

Results:
All individuals presented auditory thresholds within normal limits. The mean amplitude of the general responses of otoacoustic emissions in the right and left ears were 11.8 and 12.8 dB, respectively; the suppression effect of otoacoustic emissions was present in 73.3% for the right ear and 66.7% for the left. For brainstem auditory evoked potentials, we obtained mean wave latencies for the right and left ears respectively of wave I: 1.83 and 1.80 ms, III: 4.08 and 4.15 ms, and V: 5.96 and 6.09 ms.

Conclusions:
Individuals with neurofibromatosis type 1 present auditory thresholds within normal limits, present transient otoacoustic emissions, the nonsystematic presence of the suppression effect of otoacoustic emissions and prolonged latencies in brainstem auditory evoked potentials.