X-linked mixed hearing loss with stapes fixation is a congenital, recessive anomaly. The condition is characterized by progressive mixed hearing loss, reduced vestibular responses, and congenital ...fixation of the stapes with perilymph gusher encountered during surgical intervention. This paper describes the audiologic history of a family with two affected male children and one carrier female child. The need for early intervention and the challenges encountered in evaluation and management are discussed.
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