X-linked mixed hearing loss with stapes fixation: case reports.
نویسندگان
چکیده
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.
منابع مشابه
X-linked stapes gusher: CT findings in one patient.
A 31-year-old male patient presented with progressive mixed hearing loss since birth. A stapedectomy was attempted and was unsuccessful because of perilymph gushing. CT of the temporal bones showed bulbous dilatation of the fundi of the internal auditory canals and absence of the bone plates separating them from the base of the cochleas. This unusual abnormality was found after the attempted st...
متن کاملA Case of the Vibrant Soundbridge Stapes Coupler in Patients with Mixed Hearing Loss
The Vibrant Soundbridge (VSB) with stapes clip coupler placement at the stapes head has been used successfully to treat mixed hearing loss. Coupling between the floating mass transducer of the VSB and the stapes head is technically less demanding than incus vibroplasty and is more likely to generate a positive outcome without significantly changing residual hearing or resulting in medical or su...
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OBJECTIVES A non-progressive and conductive hearing loss with normal eardrum, but no history of trauma and infection, is highly suggestive of a congenital ossicular malformation. Among ossicular anomalies, stapes anomaly is the most common. The purpose of this study is to describe patterns of stapes anomaly and to analyze its surgical outcome with special reference to its patterns. METHODS We...
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It is often possible to elicit the stapedius reflex in patients suffering from recessive X-linked progressive mixed deafness syndrome with stapes gusher. The presence of an air-bone gap in the audiogram and the ability to elicit the stapedius reflex are conflicting. Measurements were performed on two patients who were suffering from this syndrome, to establish whether the hearing loss was of th...
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ورودعنوان ژورنال:
- Journal of the American Academy of Audiology
دوره 4 6 شماره
صفحات -
تاریخ انتشار 1993