PubMed · 10789404
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C W Cremers. 2000. Hearing: cracking the code.. https://doi.org/10.1258/0022215001903816
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Otosclerosis is a primary osteodystrophy which affects a localized area within the human temporal bone. Hearing loss is the most functional deficit caused by otosclerosis. However, tinnitus and vestibular disorders are frequently reported by otosclerotic patients, especially in those patients with inner ear involvement. The best therapy in achieving a significant improvement is surgery (stapedoplasty). In most patients if the operation is not carried out for pure middle ear type, the hearing impairment can progress to high-degree hearing loss. Recently, guidelines for the treatment of otosclerosis reported the 20 dbHL threshold as the minimum air-bone gap in performing stapedoplasty. We believe that stapedoplasty is an effective procedure for selected patients affected by otosclerosis with an air-bone gap which is smaller than 20 dbHL. An operation in the earlier phases of the disease can arrest the progression of otosclerosis, preserve inner ear structures and provide a complete auditory recovery, with increased satisfaction of the patient.
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Atypical Cogan syndrome is a systemic vascular disease close to Cogan syndrome. It is characterized by different ocular manifestations associated with Ménière-like audiovestibulatory dysfunction with symptoms arising more than 2 years before or after the ocular abnormalities. We report the case of a 63-year-old woman whose ocular manifestations involved alternating scleritis, myositis and uveitis associated with audiovestibulatory signs, and the onset of ocular muscle and nerve palsies. In the light of this observation, we discuss the clinical characteristics, diagnostic means, differential diagnoses and therapeutic modalities of this syndrome.