Round Window Electrocochleography in Genetic Causes of Hearing Loss: A Pediatric Case Series
How can hearing loss in congenitally deaf pediatric patients with cochlear implants best be measured? This is among the questions posed in ongoing research by Vivian Kaul, MD, a neurotologist and assistant professor in the Department of Otorhinolaryngology at McGovern Medical School at UTHealth Houston.
“Our goal in this study was to describe a case series of patients with common genetic mutations that result in severe, profound hearing loss. All four cases, which we evaluated and treated at a free-standing tertiary care pediatric hospital, suggested some degree of poor development of the cochlear hair cells,” Dr. Kaul says.
Interventions used in the study included genetic testing for hearing loss, round window electrocochleography (ECoG), and cochlear implantation, with cochlear microphonic responses as the main outcome measure.
“We determined that, in general, hearing loss should be measured not only through the typical formats of auditory brainstem response (ABR) evaluation and otoacoustic emissions (OAE) but also using ECoG,” she says. “ECoG can provide more sensitive data for assessing the response of the peripheral auditory system and evaluating preservation of hearing in cochlear implant users. All four cases we studied suggested significant hair cell loss compared to patients with auditory neuropathy spectrum disorder and those with normal hearing, yet provided reasonable evidence of hair cell activity that may be amenable to potential future gene therapies.”
Dr. Kaul presented the research at the national American Otological Society meeting held in Chicago in May as part of the 2024 Combined Otolaryngology Spring Meetings.