PubMed · 14174513
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C W WIESKE. 1963. HUMAN SENSITIVITY TO ELECTRIC FIELDS.. https://pubmed.ncbi.nlm.nih.gov/14174513/
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OBJECTIVE: We reviewed the results and management of cases in which total ossiculoplasty requires footplate removal. STUDY DESIGN AND SETTING: We conducted a retrospective review of 10 patients who underwent total ossiculoplasty with footplate removal between 1999 and 2002. These 10 patients represented 5.4% of those undergoing stapedectomy (n = 91) and ossiculoplasty (n = 93) during these years at a tertiary otologic referral center. RESULTS: A total of 10 patients were evaluated, with a mean 17-month follow-up. Closure of air-bone gap (ABG) to within 20 dB was achieved in 6 of 10 patients (60%). Mean postoperative ABG was 21.7 dB (500 to 4000 Hz) with an average 4-frequency pure tone improvement of 20.7 dB. Operative findings included extensive tympanosclerosis, partial obstruction of oval window by facial nerve, and changes from prior surgery. There were no patients with postoperative sensorineural hearing loss. CONCLUSIONS: The need to open the footplate in cases of total ossiculoplasty is uncommon. This may be accomplished with good improvement in hearing with minimal risk.
DFNA6/-14 is a nonsyndromic, autosomal dominant form of hearing impairment that is characterised by low-frequency sensorineural hearing loss, which in some cases is progressive. It is the only known form of dominantly inherited low-frequency hearing impairment in the Netherlands. It is caused by heterozygous non-inactivating mutations in the WFSI gene, which are also present in the Wolfram or DIDMOAD syndrome.