By Michael D. Page, MCD, CCC-A, Audiologist
Primary Children's Medical Center, Salt Lake City, UT
Presented at Patient, Family and Friends Day
Fourth International Workshop on Alport Syndrome
April 15, 1999, in Salt Lake City
Alport syndrome with its associated progressive sensorineural hearing loss presents the patient with new and increasing personal challenges. Onset of hearing loss has been reported as early as nearly ten years of age. The progressive nature of this sensorineural hearing loss provides unique requirements for amplification, and general precautions to avoid further hearing loss.
During the identification and evaluation process for hearing loss, a patient may be exposed to several methods of audiometric evaluations including otoscopy, tympanometry/acoustic reflex testing, behavioral hearing testing, brainstem auditory evoked potentials, and otoacoustic emissions.
Extent of hearing loss can be mild, moderate, severe or profound and is usually sensorineural in nature. The features of these hearing losses will be discussed briefly. Following the identification of hearing loss, amplification may be considered. New technology in hearing aids will be presented as well as the most practical options for persons with Alport syndrome.
This brief presentation will review typical audiology terms (extensive glossary of terms provided), methods of evaluations, descriptions of hearing loss types and degrees, and options for hearing aids specific to the needs of persons with Alport syndrome.