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The perilymphatic hypertension syndrome
P L Mangabeira-Albernaz1, L B Hidal, M C Iório
1Department of Otorhinolaryngology and Communication Disorders, Escola Paulista de Medicina, S. Paulo, Brasil.
Insights
Perilymphatic hypertension in children, often linked to surgical complications, causes mixed hearing loss. Despite an air-bone gap, stapedial reflexes remain present, and hearing aids offer some benefit.
Area of Science:
- Otolaryngology
- Audiology
- Pediatric Medicine
Background:
- Perilymphatic hypertension is typically associated with intraoperative complications like gushers during stapes surgery.
- Underlying conditions include modiolus defects or enlarged cochlear aqueducts, facilitating cerebrospinal fluid entry into the perilymphatic space.
- This syndrome has been identified in pediatric populations, exhibiting distinct audiological characteristics.
Purpose of the Study:
- To describe the audiological patterns associated with perilymphatic hypertension in children.
- To investigate the impact of this condition on hearing loss and auditory function.
- To evaluate the effectiveness of hearing aids in managing the hearing deficits.
Main Methods:
- Case identification of children diagnosed with perilymphatic hypertension.
- Audiological assessments including pure-tone audiometry, tympanometry, and stapedial reflex testing.
- Real-ear measurements to evaluate hearing aid performance, particularly insertion gain.
Main Results:
- Patients consistently presented with mixed hearing loss.
- An air-bone gap was observed, yet stapedial reflexes were present.
- Children demonstrated a benefit from hearing aids, but with limited low-frequency insertion gain.
Conclusions:
- Perilymphatic hypertension in children presents a recognizable audiological profile.
- The presence of stapedial reflexes despite an air-bone gap is a key diagnostic feature.
- Hearing aids can provide some auditory benefit, though low-frequency amplification may be challenging.
Abstract:
Perilymphatic hypertension is usually related to gushers which occur in the operating room when the stapes footplate is punctured or removed. These patients present defects in the modiolus, or enlarged cochlear aqueducts, which allow easy access of spinal fluid to the perilymphatic space. This syndrome was detected in children and appears to present consistent audiological patterns. They present a mixed hearing loss and in spite of the presence of an air-bone gap the stapedial reflexes are present. These children benefit from the use of hearing aids but show very little insertion gain in the low frequencies when tested with real-ear measurements.