Monitoring the Hearing Handicap and the Recognition Threshold of Sentences of a Patient with Unilateral Auditory Neuropathy Spectrum Disorder with Use of a Hearing Aid

Abstract

Introduction Treatment for auditory neuropathy spectrum disorder (ANSD) is not yet well established, including the use of hearing aids (HAs). Not all patients diagnosed with ASND have access to HAs, and in some cases HAs are even contraindicated.

Objective To monitor the hearing handicap and the recognition threshold of sentences in silence and in noise in a patient with ASND using an HA.

Resumed Report A 47-year-old woman reported moderate sensorineural hearing loss in the right ear and high-frequency loss of 4 kHz in the left ear, with bilateral otoacoustic emissions. Auditory brainstem response suggested changes in the functioning of the auditory pathway (up to the inferior colliculus) on the right. An HA was indicated on the right. The patient was tested within a 3-month period before the HA fitting with respect to recognition threshold of sentences in quiet and in noise and for handicap determination. After HA use, she showed a 2.1-dB improvement in the recognition threshold of sentences in silence, a 6.0-dB improvement for recognition threshold of sentences in noise, and a rapid improvement of the signal-to-noise ratio from +3.66 to −2.4 dB when compared with the same tests before the fitting of the HA.

Conclusion There was a reduction of the auditory handicap, although speech perception continued to be severely limited. There was a significant improvement of the recognition threshold of sentences in silence and in noise and of the signal-to-noise ratio after 3 months of HA use.

Keywords

auditory perceptual disorders - retrocochlear diseases - rehabilitation - acoustic stimulation

• References

• 1 Berlin CI, Hood LJ, Morlet T , et al. Multi-site diagnosis and management of 260 patients with auditory neuropathy/dys-synchrony (auditory neuropathy spectrum disorder). Int J Audiol 2010; 49 (1) 30-43
• 2 Sharma A, Cardon G, Henion K, Roland P. Cortical maturation and behavioral outcomes in children with auditory neuropathy spectrum disorder. Int J Audiol 2011; 50 (2) 98-106
• 3 Doyle KJ, Sininger Y, Starr A. Auditory neuropathy in childhood. Laryngoscope 1998; 108 (9) 1374-1377
• 4 Hood LJ. Auditory neuropathy/auditory dys-synchrony: new insights. Hear J 2002; 55: 17-18
• 5 Madden C, Rutter M, Hilbert L, Greinwald Jr JH, Choo DI. Clinical and audiological features in auditory neuropathy. Arch Otolaryngol Head Neck Surg 2002; 128 (9) 1026-1030
• 6 Podwall A, Podwall D, Gordon TG, Lamendola P, Gold AP. Unilateral auditory neuropathy: case study. J Child Neurol 2002; 17 (4) 306-309
• 7 Henriques MO, Costa MJ. Reconhecimento de sentenças no ruído, em campo livre, em indivíduos com e sem perda auditiva. Rev Cefac 2011; 13 (6) 1040-1047
• 8 Lima II, Aiello CP, Ferrari DV. Correlações Audiométricas do Questionário de Handicap Auditivo para Adultos. Rev CEFAC 2010; 12: 1-1
• 9 Zhang Q-J, Lan L, Shi W , et al. Unilateral auditory neuropathy spectrum disorder. Acta Otolaryngol 2012; 132 (1) 72-79
• 10 Dell'aringa AHB, Esteves MCBN, Dell'aringa AR, Arruda GV. Hearing Aid Fitting Results in a Case of a Patient with Auditory Neuropathy. Int Arch Otorhinolaryngol 2009; 13 (1) 107-110
• 11 Oliveira JRM, Kuchar J, Valarelli TP. Estratégias de Comunicação Utilizada por Indivíduos com Neuropatia/Dessincronia Auditiva. Rev CEFAC 2011; 13: 85-91
• 12 Marino MV, Mantello EB, Valadão MN, Reis ACM, Anastasio ATR. Avaliação da contribuição do aparelho de amplificação sonora no espectro de neuropatia auditiva: um estudo de caso. Rev Med 2013; 46: 36-46
• 13 Costa NTO, Martinho-Carvalho AC, Cunha MC, Lewis DR. Habilidades auditivas e comunicativas no espectro da neuropatia auditiva e mutação no gene Otoferlin: estudo de casos. J Soc Bras Fonoaudiol 2012; 24 (2) 181-187