A Retrospective Study of Hearing Loss in Patients Diagnosed with Peroxisome Biogenesis Disorders in the Zellweger Spectrum.
John Lee, Christine Yergeau, Kosuke Kawai and 2 others
PMID 34534157WHAT IT FOUND
Most patients had stable, moderately-severe to severe hearing loss, but air-bone gaps were common in young children.
Bone conduction testing is essential to distinguish true loss from middle ear fluid. Hearing aids provided significant benefit, averaging 31 to 35 dB improvement in four-frequency pure tone averages.
Key findings
01The majority of patients exhibited stable hearing thresholds over time, with air-bone gaps and transient fluctuations in air conduction suggesting middle ear disease rather than rapid progressive sensorineural loss.
02Hearing aid amplification improved four-frequency pure tone averages by an average of 31 to 35 dB in the subset of patients with available aided and unaided data.
03Air-bone gaps greater than 10 dB were evident in 61.1% of patients under 5 years of age, indicating a high prevalence of conductive components in young children with this condition.
STILL TO COME
How it was doneWhat they foundWhat it means for SLPs
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What it does not show
Bone conduction data was available for only 19 patients, leaving the type of hearing loss undetermined for the majority of the cohort. Aided and unaided data for hearing aid benefit analysis was available for only 13 patients. No patients had cochlear implant aided and unaided data collected on the same date, limiting direct comparison of implant benefit. The study is retrospective, relying on existing medical records which may lack consistent testing protocols or speech recognition data.
Declared interests
The study was supported by the National Institutes of Health (Extramural) and non-U.S. Government sources. No specific commercial conflicts of interest were declared in the provided text.
The easy way to misread this
Do not assume progressive sensorineural hearing loss is the primary feature. The data shows stable thresholds for most patients, with fluctuations in air conduction often caused by middle ear issues. Relying solely on air conduction tests may lead to incorrect diagnosis of hearing loss type and inadequate amplification fitting.