How to prevent a stapes gusher.
A stapes gusher is the result of a congenital inner ear anomaly showing at tone audiometry a conductive or mixed hearing loss. The conductive part of the hearing loss could lead to the thought to explore the middle ear. The congenital origin should lead to a high resolution. CT-scanning to evaluate a widening of the internal acoustic canal. Repeated audiometry could show especially a large conductive impairment in the lowest frequencies with a closure of the airbone gap at 2 khz and a high sensorineural high frequency loss at 4 and 8 khz. Contralateral stapedial reflexes may be present. Since the x-recessive mixed deafness syndrome (DFN3) frequently involves males with an early childhood hearing impairment, clinical suspicion should be high. When stapes surgery is considered a precise medical history is essential regarding on the start of the hearing impairment. A continuous suspicion will guide to the audiological, radiological and molecular genetic clues to trace the correct diagnosis before embarking on stapes surgery.
Cremers
C W R J
CWRJ
Department of Otolaryngology, University Medical Center St. Radboud, Nijmegen, The Netherlands.
D000293:Adolescent; D000328:Adult; D001301:Audiometry, Pure-Tone; D002648:Child; D041321:Chromosomes, Human, X; D015834:Cochlear Diseases / Q000235:genetics* / Q000517:prevention & control*; D004424:Ear Canal; D005260:Female; D005402:Fistula / Q000235:genetics* / Q000517:prevention & control*; D005808:Genes, Recessive; D006580:Genetic Carrier Screening; D020022:Genetic Predisposition to Disease / Q000235:genetics; D005820:Genetic Testing; D006314:Hearing Loss, Conductive / Q000235:genetics / Q000601:surgery; D046089:Hearing Loss, Mixed Conductive-Sensorineural / Q000235:genetics / Q000601:surgery; D006801:Humans; D007431:Intraoperative Complications / Q000517:prevention & control*; D008297:Male; D008487:Medical History Taking; D050809:POU Domain Factors / Q000235:genetics; D010375:Pedigree; D010498:Perilymph; D012665:Semicircular Canals / Q000002:abnormalities*; D012729:Sex Chromosome Aberrations; D013201:Stapes Surgery / Q000009:adverse effects*; D013577:Syndrome; D014057:Tomography, X-Ray Computed; D014722:Vestibule, Labyrinth / Q000002:abnormalities*
D050809:POU Domain Factors; C091774:POU3F4 protein, human