Int Arch Otorhinolaryngol 2014; 18 - a2447
DOI: 10.1055/s-0034-1389042

Hearing Aid Adaptation and Alport Syndrome: A Case Report

Marília Cancian Bertozzo 1, Amarilis Barreto dos Santos Andrade 1, Luiz Fernando Manzoni Lourençone 1, Valdéia Vieira de Oliveira 1
  • 1Hospital de Reabilitação de Anomalias Craniofaciais da Universidade de São Paulo

Introduction: Alport syndrome (AS) is a genetic disorder characterized by hematuria, nephritis, hearing loss, and ocular abnormalities. In males, hematuria develops in childhood, hearing loss during school years, and renal and visual impairment in the early 20s.

Objectives: To report a case of a patient treated at an institution of hearing health diagnosed with AS.

Resumed Report: A 33-year-old man patient (E.C.M.G.) was reported. At the age of 10 years was diagnosed with bilateral severe sensorineural hearing loss and adapted with bilateral hearing aids (HA) in this institution. At 18, he had hematuria; chronic nephritis was diagnosed at the age of 24 years. With 27 years, he was referred to the genetics division, where the clinical data of hearing loss, myopia of four diopters, nephritis, and normal psychomotor development leaded to the diagnosis of AS. Currently, the patient has bilateral severe sensorineural hearing loss and type A tympanometric curve uses HA effectively and was recently adapted to the microchannel model. Results show score of 100% in speech perception in silence for monosyllables in the intensity of 60 dB; without the HA and under the same conditions the score is 48%.

Conclusion: Literature shows hearing loss as one of the first AS signs, affecting 55% of males. In many cases, such as the one introduced here, its gravity hinders communication and social relationship, directly affecting quality of life. Thus, audiologic intervention has great importance regarding the hearing loss diagnosis and HA adaptation, aiming to decrease the hearing disability and improve social interaction.